Primary mediastinal (thymic) B-cell large cell lymphoma is one of the most aggressive types of large B-cell lymphoma. It most often begins in the lymph nodes of the mediastinum (the area behind the sternum, that is, behind the central flat bone in the chest to which the ribs attach). The disease quickly spreads to the entire body, and then the lymph nodes enlarge in groups on the neck, armpits, groin and other places, and single tumors also appear in the internal organs. Enlarged nodes can be felt with your hands or found using x-rays and ultrasound (ultrasound). The patient may develop such signs of illness, which are called “B-symptoms”: the temperature rises, he quickly loses weight and sweats heavily in the evening and at night. Primary mediastinal large B-cell lymphoma progresses rapidly, but is completely curable.

Diagnosis

To make a correct diagnosis, be sure to take a piece of the tumor or enlarged lymph node for morphological and immunohistochemical research. Only after such a complex comprehensive analysis can one be confident in the diagnosis and carry out effective treatment.

In order to find out exactly whether there are enlarged lymph nodes and what their number is in those parts of the body that cannot be seen during an external examination, it is necessary to do a computed tomography scan of the chest, abdomen and pelvis. The stage of the disease is determined according to the system Ann Arbor, which shows which organs and lymph nodes are affected by the disease.

Treatment

To cure a patient with this disease, a variety of regimens are used using medicine "rituximab". Treatment depends on the characteristics of the disease and, most importantly, on the general health of the patient. Between courses of chemotherapy, a control CT scan may be required to assess their effectiveness. If treatment with small doses of drugs does not help, then more intensive treatment is carried out, the so-called

Catad_tema Blood cancer - articles

Diffuse large B-cell lymphoma in adults. Clinical recommendations.

Diffuse large B-cell lymphoma in adults

ICD 10: S83.3

Year of approval (revision frequency): 2016 (reviewed every 2 years)

ID: KR129

Professional associations:

• National Hematological Society Russian Professional Society of Oncohematologists

Approved

At the XIII Russian conference with international participation “Malignant lymphomas” in 2016.

Agreed

Scientific Council of the Ministry of Health of the Russian Federation__ __________201_

1st line therapy

2nd line therapy

Radiation therapy

Auto-TSKK

List of abbreviations

DLBCL – diffuse large B-cell lymphoma

CT - computed tomography

MRI – magnetic resonance imaging

Ultrasound – ultrasound examination

RT – radiation therapy

HDCT with auto-HSCT is high-dose chemotherapy followed by transplantation of autologous hematopoietic stem cells.

PMBCL - primary mediastinal large B-cell lymphoma

CNS - central nervous system

PET – positron emission tomography

IFT - immunophenotyping

Terms and Definitions

Primary mediastinal large B cell lymphoma (PLCL) refers to primary extranodal tumors and originates from B cells of the medulla of the thymus.

Diffuse large B cell lymphoma (DLBCL) is a heterogeneous group of lymphatic tumors with different clinical, morphological, immunophenotypic, cytogenetic manifestations and with different responses to therapy. The tumor substrate is large B-lymphoid cells with pronounced atypia and polymorphism, with a nuclear size two or more times greater than the size of the nucleus of a small lymphocyte. Tumor cells in most cases are located diffusely, but can also be scattered among mature B-lymphocytes, sometimes against the background of a T-cell environment, or form focal clusters.

Nodal DLBCL– a disease with primary and predominant damage to the lymph nodes

Extranodal DLBCL– a disease with primary damage to any organ except lymph nodes

1. Brief information

1 . 1 Definition

DLBCL is a heterogeneous group of lymphatic tumors with different clinical, morphological, immunophenotypic, cytogenetic manifestations and different responses to therapy. The tumor substrate is large B-lymphoid cells with pronounced atypia and polymorphism, with a nuclear size two or more times greater than the size of the nucleus of a small lymphocyte. Tumor cells in most cases are located diffusely, but they can also be scattered among mature B-lymphocytes, sometimes against the background of a T-cell environment, or form focal clusters.

1.2 Etiology and pathogenesis.

B cells are formed in the bone marrow, where the primary rearrangement of genes encoding the synthesis of immunoglobulins occurs. Genes of the variable region of light chains (k or?) are assembled using connecting (J – joing), and genes of the variable region of heavy chains are assembled using connecting (J) and diverse (D – diversity) segments.

Cells with “successful rearrangement” of immunoglobulin genes (naïve B cells) leave the lymphoid tissue and enter secondary lymphoid organs - lymphoid organs, tonsils, spleen, Peyer’s patches. In them, B cells exposed to antigen form follicles along with follicular dendritic cells and T cells. As a result of this process, germinal centers are formed in secondary lymphoid follicles. In the germinal center of secondary follicles, naive B cells, lacking adequate antigen and unable to produce a functional antibody, undergo apoptosis, i.e. die. But in B cells with adequate antigens and capable of producing a functional antibody in the germinal center, an immunoglobulin class switch occurs (IgM, IgD to IgG, IgA or IgE), as well as somatic hypermutation (replacement of one nucleotide in the hypervariable regions of immunoglobulins), after which B- the cells leave the follicle to become terminally differentiated plasma cells or long-lived memory B cells. Random failures in the control of these processes play a decisive role in the development of B-cell tumors, including DLBCL.

The pathogenesis of DLBCL is poorly understood. The diversity of clinical and morphological manifestations of DLBCL and immunophenotype suggests that DLBCL is not a single nosological form, but a group of lymphatic tumors with similar but different pathogenesis.

This is also proven by molecular cytogenetic studies in recent years, which have made it possible to identify several variants of DLBCL depending on the level of differentiation of tumor cells, types of disruption of cellular processes, and chromosomal abnormalities.

Many genes that regulate events in the germinal centers are likely to be important in the pathogenesis of DLBCL, but the mechanism studied is gene rearrangement Bcl-6, which is caused by improper immunoglobulin class switching of B cells in the germinal center. Gene Bcl-6 located at locus 3q27 and expressed exclusively by germinal center B cells.

Under physiological conditions, the gene Bcl-6 binds to certain regulatory DNA sequences and affects the transcription of other genes involved in B-cell activation and terminal differentiation of lymphocytes. When the 3q27 locus is rearranged, further differentiation of B cells into plasma cells is blocked, which leads to uncontrolled proliferation of germinal center B cells.

1.3 Epidemiology.

Diffuse large B-cell lymphoma is one of the most common types of lymphoproliferative diseases in adults (30-50% of all non-Hodgkin lymphomas). At the age of 18 years, the frequency of this variant of aggressive B-cell tumor does not exceed 8-10%. The incidence of DLBCL averages 4-5 per 100,000 population per year. The risk of developing a tumor increases with age and is significantly higher in people who are seropositive for the hepatitis C virus, in the presence of the human immunodeficiency virus (HIV), and in autoimmune diseases. Men and women suffer from DLBCL at approximately equal rates.

1.4 Coding according to ICD 10:

C83.3 Large cell lymphoma (diffuse) - reticulosarcoma

1.5 Classification.

Primary mediastinal DLBCL

Primary DLBCL of the central nervous system

Intravascular DLBCL

Lymphomatoid granulomatosis

Primary DLBCL of serous cavities

Primary cutaneous DLBCL, lower extremity type

DLBCL associated with chronic inflammation

EBV positive DLBCL, (NOS)

DLBCL with T-cell predominance

EBV positive mucosal and skin ulcer

ALC positive DLBCL

Plasmablastic DLBCL

HHV8 positive DLBCL, NOS

Large cell lymphoma with IRF4 rearrangement

B-cell lymphoma, unclassified, with features of DLBCL and classic Hodgkin lymphoma

1.6 Clinical signs

They are varied, nonspecific and are determined by the primary localization, growth rate, extent of the tumor, as well as the presence of symptoms of intoxication.

2. Diagnostics

2.1 Complaints and anamnesis

when did certain signs of the disease first appear;

are there B-symptoms (sweating, weight loss, fever 38.0 C or higher);

what treatment was given; whether there was therapy with glucocorticosteroids and/or other cytostatic drugs, as well as monoclonal antibodies;

Is there a family member with lymphoma?

Comments:collection of complaints and medical history will allow us to determine the nature of the disease (primary DLBCL or transformation of indolent lymphoma). With DLBCL, complaints can be varied depending on the location of the tumor: brain, mediastinum, intestine, stomach, orbit, kidney, spleen, and so on. Given the aggressive nature of the disease, the medical history is usually short.

2.2 Physical examination of patients with DLBCL does not differ from that for diseases of internal organs.

2.3 Laboratory diagnostics

Recommended perform all routine general therapeutic tests: clinical blood test with determination of hemoglobin, red blood cells, platelets, leukocytes, counting the leukocyte formula and reticulocyte count; biochemical blood test, general urine test, coagulogram, electropheresis of serum proteins, determination of markers of viral hepatitis B and C and HIV, direct Coombs test.

A comment: a biochemical blood test includes the mandatory determination of the following parameters - LDH, uric acid, urea, creatinine, total protein, albumin, bilirubin, AST, ALT, alkaline phosphatase, electrolytes, calcium. A direct Coombs test is performed if there are clinical or laboratory signs of hemolysis

Comments: The diagnosis of DLBCL is established only on the basis of a morphological examination of a biopsy specimen. Morphological examination is carried out using histological and immunohistochemical methods. In some cases, cytological, molecular biological and genetic tests are necessary. A single cytological examination of punctates or smears of lymph nodes or other tumor foci is not a sufficient basis for nosological verification of lymphomas. In exceptional cases (localization of the tumor in hard-to-reach anatomical areas), the object of study may be tissue material obtained using a “pistol” (“core”) biopsy.

Comments: Morphological examination of bone marrow punctate (sternal or other) does not replace histological examination of trephine biopsy specimen.

Comments:performing IFT is mandatory in the presence of lymphocytosis in a general blood test (regardless of the number of leukocytes), or in the myelogram, as well as in the predominance of lymphoid cells, atypical lymphocytes or cells with blast morphology in pleural, ascitic or other biological fluids. Performing IFT allows for a quick differential diagnosis of tumor and reactive lymphocytosis, which is important for determining further tactics for examining the patient. The material for IFT can be blood cells, bone marrow, effusion fluids, cerebrospinal fluid, homogenized tissue samples (spleen, lymph nodes, etc.), cell suspension obtained by fine-needle aspiration of lymph nodes.

Comments:if there is no neuroleukemia, then the lumbar puncture is not repeated.

Recommended in case of relapse or progression of the disease, it is necessary to perform a repeat biopsy and morphological examination of the affected lymph nodes or foci located extranodal. .

Comments:A repeat biopsy will clarify the morphological variant of relapse/progression and exclude non-tumor lesions (for example, tuberculosis, fungal invasion, a second tumor). A repeat biopsy is also indicated, in some cases, in the presence of residual lesions to confirm or rule out remission.

Recommended repeated aspiration and trephine biopsy of the bone marrow for routine assessment of treatment results (in case of initial bone marrow damage) and in the event of the appearance of clinically unmotivated cytopenia and fever (regardless of the presence of the original lesion).

Comments: Bone marrow aspirate may be informative for assessing regeneration and dysplastic changes in myelopoiesis. In patients with DLBCL with bone marrow lesions, cytological examination of punctate tissue to assess changes in the volume of tumor infiltration is not informative.

2.4 Instrumental diagnostics

Recommended perform a CT scan of the brain, neck, chest, abdominal and pelvic organs, x-ray of the chest organs in two projections (if it is not possible to perform a CT scan), ultrasound of peripheral lymphatic, intra-abdominal and retroperitoneal nodes, abdominal and pelvic organs (if it is not possible perform a CT scan of the indicated areas), ECG and Echo-CG, endoscopic examination of the stomach and intestines if the patient has complaints indicating their damage.

A comment: Ultrasound can be used to monitor treatment, but is not the standard for establishing the stage of the disease and assessing the effectiveness of treatment

2.5 Additional research, specialist consultations

If indicated, additional research methods may be performed:

Comments:bone scintigraphy - if necessary to exclude bone lesions, CT/MRI of the brain - if necessary to exclude damage to the central nervous system, PET - if it is impossible to determine lesions by another method

2.6. Staging, formulation of diagnosis

Staging of DLBCL is carried out according to the classification Ann-Arbor (Table No. 1).

Stage	Criteria
	Involvement of one group of lymph nodes
	Focal involvement of one segment or area of ​​a non-lymphatic organ
	Involvement of more than one group of lymph nodes located on one side of the diaphragm
	Focal involvement of one segment or area of ​​a non-lymphatic organ with its regional lymph nodes, with or without involvement of other lymph nodes located on the same side of the diaphragm
	Involvement of more than one group of lymph nodes located on one side of the diaphragm, combined with involvement of the spleen
	Damage to groups of lymph nodes located on both sides of the diaphragm
	Damage to groups of lymph nodes located on both sides of the diaphragm, in combination with local damage to a non-lymphatic organ
	Damage to groups of lymph nodes located on both sides of the diaphragm, combined with damage to the spleen
	Diffuse or disseminated damage to one or more non-lymphatic organs or tissues with or without damage to regional and distant lymph nodes, or isolated damage to a non-lymphatic organ involving distant lymph nodes in the pathological process

The formulation of the diagnosis should indicate: what is affected, the patient’s condition on the ECOG scale, the International Prognostic Index (IPI), the stage of the disease.

3. Treatment

3.1 First-line chemotherapy.

3.1.1LocalI- IIstages.

3.1.2 Common III-IV stages

Comments: For elderly patients with severe concomitant pathology and/or severely weakened somatic status, the use of less toxic immunochemotherapy regimens, for example, R-miniCHOP, is recommended.

In the presence of serious cardiac pathology (decrease in left ventricular ejection fraction below 50%), regimens that do not contain doxorubicin can be used - R-CEPP, R-CEOP, R-GCVP, etc.

3.2 Second-line chemotherapy.

Comments: second line modes: DHAP, ESHAP, ICE, GDP, GemOx, MINE and others. Rituximab should be used with any of these regimens if relapse was diagnosed more than 6 months after the previous drug administration. Elderly patients or patients with comorbidity who are not candidates for transplantation can be prescribed the same second-line regimens or other regimens: R-GemOx, R-B, R-CEPP, DA-EPOCH-R, metronomic PEPC regimen. Treatment tactics for recurrent DLBCL after autoHSCT should be determined individually depending on previously received antitumor regimens and the general condition of the patient. Inclusion in clinical trials may be preferable, including for patients who are not candidates for high-dose chemotherapy with auto-HSCT. In young patients with good somatic status, allogeneic HSCT can be considered.

3.3 Surgical treatment.

There is no surgical treatment for DLBCL. Only in cases of primary damage to the spleen and in the absence of lesions accessible for biopsy, splenectomy is indicated. In all other cases, surgical intervention should be limited to a biopsy of the lesion, as well as within the framework of providing assistance in life-threatening conditions.

Comments. The main indication for diagnostic, and at the same time therapeutic, splenectomy is the presence of a hypoechoic lesion/foci in the spleen parenchyma that accumulates a contrast agent upon intravenous administration. In this case, the size of the spleen can sometimes be normal or slightly enlarged.

3.4 Radiation therapy

3.4.1 Local I - II stages

Recommended I with a consolidation goal after systemic chemotherapy in a total dose of 30 Gy; in case of partial response to drug treatment, RT as a treatment option at a dose of 36-40 Gy; with significant amounts of residual disease after chemotherapy, adding a local “boost” up to 40-45 Gy; in case of inability to perform HPCT (elderly or patients with concomitant diseases), upon achieving a stable antitumor effect with the aim of consolidation at a dose of 40-46 Gy.

3.4.2 Common III - IV stages

Recommended when, after completion of chemotherapy, regression of any single residual lesion can make it possible to convert partial remission into complete or stabilization into partial remission. .

3.5 Primary mediastinal (thymic) large B-cell lymphoma

Recommended As the first method of antitumor treatment, various immunochemotherapy regimens can be used: R-CHOP, MACOP-B+R, DA-EPOCH-R. If partial remission is not achieved and the tumor has high metabolic activity, perform HDCT with auto-HSCT with possible subsequent radiation therapy for the residual tumor.

Recommended conduct radiation therapy: after a complete metabolic response to chemotherapy as a consolidation dose of 30 Gy to the areas of the initially affected lymph nodes; in the presence of a PET-positive residual tumor on the areas of the original lesion in a dose of 30 Gy + “boost” on the residual tumor of 6-10 Gy. .

Comments: lymph nodes of the roots of the lungs are included in the irradiation volume only if their damage is indicated; it is advisable to perform 3D CRT with respiratory gating to reduce pulmonary and cardiac toxicity

3.6 Maintenance therapy

4. Rehabilitation

There are no special rehabilitation methods for DLBCL. Rehabilitation in the event of complications during the disease and treatment is carried out within the framework of the appropriate nosologies. It is recommended to lead a healthy lifestyle, avoid excessive sun exposure and thermal physiotherapy.

5. Prevention and clinical observation

There are currently no methods for preventing DLBCL, since the etiological factor(s) leading to the development of the disease are unknown. Clinical observation by a hematologist or oncologist is carried out during the treatment period and after achieving remission of the lymphoma.

5.1 Prevention of neuroleukemia.

Recommended in the early stages of therapy for all patients with a high risk of CNS involvement and includes 4 intrathecal injections of methotrexate (15 mg), cytarabine (30 mg), dexamethasone (4 mg) and 2 systemic injections of high doses of methotrexate (1.5-3 g/m2) . .

Comments: one intrathecal injection on the 1st day of courses 1-4, methotrexate in high doses can be administered intravenously on the 15th day of courses 1 or 2 and after completion of treatment.

6. Additional information affecting the course and outcome of the disease

6.1 Errors and unreasonable assignments

Comments:the diagnosis of lymphoma cannot be established by puncture, without a full histological and histochemical examination

Comments:bone marrow lesions cannot be diagnosed by puncture, without a full histological and histochemical examination

6.2 Lymphomas and pregnancy

Recommended Discuss with all patients of childbearing age of both sexes the possibility of cryopreservation of sperm or ovarian tissue before starting therapy. The need for hormonal protection against pregnancy, as well as possible methods of hormonal protection of the ovaries during intensive treatment programs, should be discussed with women of childbearing age.

Comments:chemotherapy and radiation to the pelvic area can lead to irreversible sterility of the patient

Comments:If lymphoma (usually primary mediastinal large B-cell) is detected in the early stages of pregnancy, it is necessary to carry out chemotherapy with the exception of chemotherapy drugs that penetrate the placental barrier and bring the pregnancy to 32-36 weeks, and continue chemotherapy after birth.

6.3 Forecast

The prognosis depends on the extent of the tumor, the presence or absence of signs of an unfavorable prognosis, the chemosensitivity of the tumor, as well as a complete and timely diagnosis and the use of adequate treatment tactics.

Criteria for assessing the quality of medical care

Quality criteria

Level of evidence

A tissue biopsy of the lymph node (the lesion) and a morphological (cytological) and immunohistochemical study were performed

A detailed general (clinical) blood test was performed

A biochemical blood test was performed (a study of the level of total protein, albumin, urea, creatinine, uric acid, alanine aminotransferase, aspartate aminotransferases, total and direct bilirubin, cholesterol, lactate dehydrogenase, alkaline phosphatase, calcium, blood electrolytes)

Computed togography and/or ultrasound and/or magnetic resonance imaging of the chest and abdomen and pelvis and additional lesions were performed

Positron emission tomography was performed

A trepanobiopsy and morphological (histological) examination of a bone marrow specimen was performed

Electrocardiography performed

A coagulogram was performed (indicative study of the hemostatic system)

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Appendix A1. Composition of the working group

Aslanidi Irakliy Pavlovich. Doctor of Medical Sciences, Professor, Head of the Nuclear Diagnostics Department of the Federal State Budgetary Institution Scientific Center for Cardiovascular Surgery named after. A.N. Bakulev"

Baykov Vadim Valentinovich. Doctor of Medical Sciences, Professor of the Department of Pathological Anatomy, Head of the Pathomorphology Laboratory of the Research Institute of Children's Oncology, Hematology and Transplantology named after. R.M. Gorbacheva First St. Petersburg State Medical University named after. acad. I.P. Pavlova, St. Petersburg

Vinogradova Yulia Nikolaevna. Doctor of Medical Sciences, radiotherapist, leading researcher at the Department of Radiation Therapy for Systemic Diseases, heads the Department of Radiology and Surgical Technologies, Russian Scientific Center for Radiology and Surgical Technologies, Ministry of Health of the Russian Federation, St. Petersburg

Demina Elena Andreevna. Doctor of Medical Sciences, Professor, Oncohematologist, Leading Researcher at the Department of Chemotherapy for Hemoblastosis, Federal State Budgetary Institution Russian Oncological Research Center named after. N.N. Blokhin" of the Ministry of Health of Russia, Moscow.

Ekaeva Irina Viktorovna, Ph.D., Department of Nuclear Diagnostics, Federal State Budgetary Institution Scientific Center for Cardiovascular Surgery named after. A.N. Bakulev"

Ilyin Nikolai Vasilievich. Doctor of Medical Sciences, Professor, radiotherapist of the highest category, head of radiotherapy department No. 1 of the Russian Scientific Center of Radiology and Surgical Technologies, Ministry of Health of the Russian Federation, St. Petersburg

Katunina Tatyana Anatolevna, Candidate of Medical Sciences, Department of Nuclear Diagnostics, Federal State Budgetary Institution Scientific Center for Cardiovascular Surgery named after. A.N. Bakulev"

Kovrigina Alla Mikhailovna. Doctor of Biological Sciences, pathomorphologist, professor of the Department of Pathological Anatomy, Cytology and Molecular Pathology, IPK FMBA RF, Head of the Pathoanatomical Department of the Federal State Budgetary Institution "Hematological Research Center" of the Ministry of Health of the Russian Federation, Moscow

Kravchenko Sergey Kirillovich. Candidate of Medical Sciences, hematologist, associate professor, head of the scientific and clinical department of chemotherapy of hematological malignancies of the Federal State Budgetary Institution Hematological Research Center of the Ministry of Health of the Russian Federation, assistant of the Department of Hematology and Transfusiology of the State Budgetary Educational Institution of Further Professional Education RMAPO, Moscow

Krivolapov Yuri Alexandrovich. Doctor of Medical Sciences, Professor, Pathologist, Head of the Department of Clinical Molecular Morphology, North-Western State Medical University. I. I. Mechnikova, St. Petersburg

Levshin Nikolai Yurievich. Candidate of Medical Sciences, Associate Professor of the Department of Polyclinic Therapy and Clinical Laboratory Diagnostics, Yaroslavl State Medical Academy, Yaroslavl

Matsionis Alexander Eduardovich. Doctor of Medical Sciences, Academician of the Russian Academy of Natural Sciences, pathomorphologist of the highest category, head of the laboratory of immunomorphology of the State Budgetary Institution of the Rostov Region "Pathological-Anatomical Bureau", Rostov-on-Don.

Mikhailova Natalya Borisovna. Doctor of Medical Sciences, hematologist, head of the department of clinical oncology (chemotherapy) of the Research Institute of Children's Oncology, Hematology and Transplantology named after. R.M.Gorbacheva, St. Petersburg

Mukhortova Olga Valentinovna. Doctor of Medical Sciences, radiologist of the highest category, senior researcher at the Department of Nuclear Diagnostics (PET Center) at the Scientific Center for Cardiovascular Surgery named after. A.N. Bakuleva, Moscow

Osmanov Evgeny Alexandrovich. Professor, Doctor of Medical Sciences, doctor of the highest qualification category, head of the department of chemotherapy of hematological malignancies of the Russian Research Center of the Russian Academy of Medical Sciences, Moscow

Poddubnaya Irina Vladimirovna. Corresponding Member of the Russian Academy of Sciences, Honored Education Worker of the Russian Federation, Doctor of Medical Sciences, Professor, Head of the Department of Oncology, Vice-Rector for Academic Affairs and International Cooperation, Russian Medical Academy of Postgraduate Education (RMAPO), Ministry of Health of the Russian Federation, Moscow

Popova Marina Olegovna. Candidate of Medical Sciences, hematologist at the Institute of Pediatric Hematology and Oncology named after. R.M. Gorbachev, St. Petersburg

Roitman Evgeniy Vitalievich. Doctor of Biological Sciences, Professor of the Department of Hematology, Oncology and Radiation Therapy, Russian National Research Medical University named after. N.I.Pirogov Ministry of Health of Russia, Moscow

Sotnikov Vladimir Mikhailovich. Doctor of Medical Sciences, Professor, Radiologist, Oncologist of the highest category, Chief Researcher, Head of the Laboratory of Radiation Therapy, Research Department of Innovative Technologies in Radiotherapy and Chemoradiation Treatment of Malignant Tumors, Federal State Budgetary Institution Russian Scientific Center for Radiotherapy, Ministry of Health of the Russian Federation, Moscow

Trofimova Oksana Petrovna. Doctor of Medical Sciences, radiologist, leading researcher at the Radiological Department of the Russian Cancer Research Center named after. N.N. Blokhina, Moscow

Tumyan Gayane Sergeevna. Doctor of Medical Sciences, oncologist, leading researcher at the Department of Chemotherapy for Hemoblastosis, Federal State Budgetary Institution "Russian Cancer Research Center named after. N.N. Blokhin", Professor, Department of Oncology, Russian Medical Academy of Postgraduate Education, Moscow

Shmakov Roman Georgievich. Doctor of Medical Sciences, Chief Physician of the Federal State Budgetary Institution "Scientific Center of Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov" Ministry of Health of the Russian Federation, Moscow

Hematologists

Oncologists

Evidence collection methodology

Methods used to collect/select evidence:

Search for publications in specialized periodicals with an impact factor > 0.3;

Search in electronic databases.

Databases used for collecting/selecting evidence:

Methods used to analyze evidence:

Systematic reviews with evidence tables.

Methods used for quality and strength of evidence:

Expert consensus;

Assessing the significance of evidence in accordance with the evidence rating scheme (Tables 3,4).

Tables 3,4

Levels and degrees of evidence

Level of evidence	Type of evidence
	At least one large randomized controlled trial of high quality (low error rate) or meta-analysis of homogeneous randomized trials of high quality
	Small randomized trials or large randomized trials with a high probability of error. Meta-analysis of similar studies or meta-analysis of heterogeneous studies
	Prospective cohort studies
	Retrospective cohort or case-control studies
	Studies without a control group, individual cases, expert opinion

Good Practice Points (GPPs):

External expert assessment;

Internal expert assessment.

These draft guidelines were peer reviewed by independent experts who were asked to comment on the quality of the interpretation of the evidence and the development of the recommendations. An expert assessment of the presentation of the recommendations and their accessibility to understanding was also carried out.

The recommendations were discussed and approved by leading specialists from specialized Federal Centers of the Russian Federation and practical doctors. The draft clinical guidelines were reviewed at meetings of the working group in 2015-2016, at the Expert Forum on the diagnosis and treatment of malignant lymphoproliferative diseases “Lymforum”, the annual Russian conference with international participation “Malignant Lymphomas”, as well as the III Congress of Russian Hematologists.

Final edition:

For final revision and quality control, the recommendations were re-analyzed by members of the team of authors, who came to the conclusion that all significant comments and comments from experts were taken into account, and the risk of systematic errors in development was minimized.

Appendix B. Patient management algorithms

Appendix B: Patient Information

Diffuse large B-cell lymphoma is an aggressive lymphoproliferative disease. The prognosis depends on many factors, including the extent of the lesion, and therefore a complete and comprehensive examination is necessary.

Timely and adequate treatment is the key to success. Therefore, the patient must strictly follow all the doctor’s instructions regarding the time and timing of diagnostic procedures, as well as chemotherapy courses.

Be sure to report adverse events, if any, during treatment.

The clinical picture consists of symptoms of compression or invasion of the mediastinal tumor into neighboring organs.

Forms of lymphoma

The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma develops when immune system cells called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Malignant lymphocytes can travel to many parts of the body, including lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. There are two main types of lymphocytes that can develop into lymphoma: B lymphocytes (B cells) and T lymphocytes (T cells).

B-cell lymphomas are much more common than T-cell lymphomas and account for about 85 percent of all non-Hodgkin lymphomas (NHL). Diffuse B-cell lymphomas (DCL) are the most common form of NHL, accounting for about 30 percent of newly diagnosed cases. CCL occurs in both men and women, although it is more common in men. Diffuse large B-cell lymphomas (DLBCL) can occur in childhood. Approximately half of patients are over 50 years of age.

DLBCL is an aggressive (fast-growing) lymphoma that can occur in the lymph nodes or outside the lymphatic system, in the gastrointestinal tract, testes, thyroid, skin, breast, bones, or brain.

Often the first sign of DLBCL is painless, swelling in the neck, armpit, or groin area. For some patients, the swelling may be painful. Symptoms may include night sweats, fever and unexplained weight loss. Patients may notice fatigue, loss of appetite, shortness of breath, or pain.

Types of DLBCL

There are several subtypes of DLBCL that can affect a patient's prognosis and treatment options. For example, mediastinal lymphoma (MCL), which affects only the brain, is called primary central nervous system lymphoma and is treated differently than DLBCL, which affects areas outside the brain.

Another example is primary mediastinal B-cell lymphoma, which often occurs in young patients and grows rapidly in the chest (mediastinal lymphoma).

Most cases do not fall into one of these categories; they are considered diffuse mediastinal B-cell lymphoma. These subtypes are named according to their cells of origin and include B-cell germinal centers (BCGs) and activated B-cell-like (ABCs). These groups of patients may have different prognoses when treated.

Additionally, a related type of aggressive lymphoma is called "Double Whammy" lymphoma exhibits specific genetic abnormalities that can affect the outcome. Use of this information has the potential to change treatment and is under active investigation.

Diagnosis and staging

Tissue biopsy is necessary for a definitive diagnosis of DLBCL. A biopsy is a minor surgical procedure to remove part or all of a diseased lymph node or other abnormal area. The operation is performed under local or general anesthesia.

Once the diagnosis of DLBCL is confirmed, the next step is to find lesions in the body. Because BCL is a cancer of the blood, the entire body must be examined to find all lymphomas. This is usually done using computed tomography (CT) or positron emission tomography (PET)/CT. A bone marrow biopsy is done to look for lymphoma cells in the bone, and sometimes a spinal tap is done to determine if there are lymphoma cells in the brain and spinal cord.

The doctor will use the results of these tests to evaluate the stage of the lymphoma. Limited stage disease represents lymphoma affecting only one area of ​​the body, while advanced stage disease indicates that the lymphoma has spread to multiple organs.

Treatment Options

Because BCL can spread quickly, it requires immediate treatment. A combination of chemotherapy and the monoclonal antibody rituximab (Rituxan), with or without radiation therapy, can lead to disease remission in a large number of patients with this form of lymphoma. The most widely used treatment for DLBCL is R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), which is usually given in 21-day cycles. Sometimes another chemotherapy drug, etoposide, is added to the R-CHOP regimen, resulting in a drug combination called R-EPOCH. For many patients, initial treatment is effective and the BCL does not return after treatment.

However, for patients whose disease progresses (does not respond to treatment) or relapses (comes back after treatment), secondary therapies may be successful. Chemotherapy in combination with stem cell transplantation can be used to treat patients with DLBCL who relapse after initial chemotherapy.

Most patients who undergo a stem cell transplant will receive their own stem cells (autologous stem cell transplant). Sometimes, the patient receives stem cells from a donor (allogeneic stem cell transplantation). Patients who are not candidates for a stem cell transplant, or who choose not to have a stem cell transplant, can have different combination chemotherapy regimens.

Bendamustine (Treanda) plus rituximab, rituximab monotherapy, lenalidomide (Revlimid) plus rituximab, and gemcitabine combinations are secondary treatments that may also be used, although none of these agents or regimens have been indicated for patients with DLBCL. Combination therapies are currently being tested in clinical trials for the treatment of newly diagnosed and relapsed DLBCL patients:

• Brentuximab vedotin.
• Chimeric antigen receptor therapy (CAR-T).
• Ibrutinib.
• Lenalidomide.

Clinical trials are investigating the use of these agents at various stages of treatment, as well as in specific groups, including newly diagnosed patients, patients with relapsed/refractory disease, and older adults with specific molecular subtypes.

For example, because patients with lymphoma of the GCB subtype may have a better response to standard R-CHOP chemotherapy than those with the ABC subtype. Researchers are studying new treatments that improve treatment for patients with ABC DLBCL. Clinical trials studying these drugs are in various phases of development. It is very important to remember that today's scientific research is constantly evolving. Treatment options may change as new treatments are introduced. Modern treatments are improving, so it is important that patients talk to their doctor about new treatments.

What should you know about mediastinal lymphoma?

One of the types of non-Hodgkin lymphomas are malignant tumors located in the chest cavity.

They are localized in the space of the rib - spine - diaphragm - sternum.

This group of diseases got its name from its anatomical feature – mediastinal lymphoma.

Types of disease

The main division of oncological lesions of the mediastinum is carried out according to the cause of origin, such as:

Primary malignant lesions of the mediastinal lymph system most often manifest as indolent (slow-growing) non-Hodgkin lymphomas of the B-cell type.

The presumptive cause is considered to be primary degeneration (in the lymph nodes) or a violation of the formation of lymphocytes in the mediastinal organs.

Aggressive forms (T-cell) of lymphatic lesions of the mediastinum are quite rare and manifest themselves by spreading into adjacent anatomical areas, in particular, they can be affected:

Secondary malignant tumors are metastases that form as a consequence of an oncological process occurring in another organ.

The manifestation of secondary malignant foci of the disease is associated with the characteristics of lymph movement in the area of ​​the affected organ. The most typical secondary manifestation is malignant lung disease - it forms metastases in 80 out of 100 cases.

The next most common cause of secondary lesions is considered to be malignant lesions of the digestive tract.

Why do malignant lesions occur?

As with any cancer, the causes of lymphomas in the mediastinum have not been determined. However, as a result of the research and statistical analysis, risk factors were identified, which include:

• violation of the general environmental situation;
• problems of rational nutrition and environmental friendliness of products;
• radiation exposure (including for medicinal purposes)
• carcinogenic substances (in production);
• pesticides and herbicides (in agriculture);
• the presence in the body of viruses that suppress and disrupt the functioning of the immune system (HIV or Epstein-Barr virus);
• autoimmune diseases, both congenital and acquired;
• age criterion.

It should be noted that mediastinal lymphoma affects men 30% more often than women.

Signs of the disease

Mediastinal lymphoma has manifestations common to all malignant neoplasms, which include:

• unmotivated loss of appetite;
• weight loss;
• disturbances in the functioning of the nervous system, expressed in increased irritability and apathy;
• increased temperature for no apparent reason;
• increased sweating at night;
• decreased body immunity.

A decrease in the level of the body’s immune defense naturally leads to the emergence of infectious diseases of various types:

Specific signs

In addition to general symptoms, malignant lesions of the mediastinum are characterized by specific symptoms. They are associated with the localization of the lesion.

If the respiratory system is affected, symptoms typical of malignant lung disease are observed:

• pain in the sternum during breathing;
• dyspnea;
• cough with sputum with blood clots.

Damage to the digestive system is often accompanied by dysphagia - difficulty swallowing.

In the case of involvement of the vertebral parts of the spinal cord, a characteristic manifestation of the lesion is radicular syndrome, typical of a vertebral hernia, expressed by the following symptoms:

• partial or complete loss of sensation in the limbs;
• significant decrease in motor activity;
• the occurrence of various problems with the pelvic organs;
• partial or complete paresis and paralysis.

Diagnosis of cancer

Non-Hodgkin's lymphomas, which include mediastinal lymphoma, are quite difficult to detect. So complicated that it turns into a long and labor-intensive process, which includes a lot of diagnostic methods.

A typical diagnostic scheme for this disease is as follows:

• initial examination of the patient;
• palpation of lymph nodes;
• complete blood count;
• Ultrasound of all internal organs;
• CR and MRI;
• X-ray of the respiratory system;
• endoscopic examination of the larynx;
• tumor biopsy and subsequent histological and immunological examination of the sample;
• bone marrow biopsy (to exclude leukemia as a diagnosis);
• spinal (lumbar) puncture (examination of cerebrospinal fluid for the presence of malignant cells).

The most informative is a histological analysis of a sample of tumor tissue - it allows you to determine the degree of malignancy of the formation and the stage of development. This information is necessary to plan the most effective treatment measures.

Therapy methods

The accepted method of treatment for primary oncological lesions of the mediastinum is systemic chemotherapy. As a result, the medicinal effect extends to the entire body as a whole, covering the entire lymphatic system of the patient.

Surgery or radiation therapy for lymphatic lesions of the mediastinum is used only for direct medical reasons and is in this case an auxiliary means.

Treatment success

For patients with mediastinal lymphosis, the prognosis for successful treatment depends on many factors, including:

• stage of the disease;
• the degree of pathological damage to the lymphatic system;
• location of the focus of oncological manifestation;
• state of the immune system;
• age group of the patient.

For non-aggressive forms in the initial stage, the prognosis is quite favorable, with patient survival (10-year period covered) of about 90%.

If malignant cells penetrate the bone marrow or spread through the circulatory system, the rates drop to 20% for a period of several years.

Development of mediastinal lymphoma of the mediastinum

The mediastinum is the area in the middle of the chest, covered by the sternum and the spinal column. In the mediastinum area there are many important organs, systems and vessels: the heart, aorta, trachea, esophagus, vena cava, regional bronchial lymph nodes and much more.

Mediastinal lymphoma is a malignant tumor formation that forms in the bronchomediastinal lymph nodes. During the development process, the tumor affects neighboring lymph nodes and nearby organs.

Types and stages

All lymphomas are divided into these subtypes:

1. Primary. Lymphoma is formed from the tissue of a lymph node located in the mediastinal (intrathoracic) network of lymph nodes and becomes the primary malignant focus.
2. Secondary, also called metastatic. Lymphoma develops after tumor cells enter the area of ​​the mediastinal lymph nodes (through blood or lymph), the source of which may be any primary malignant neoplasm located in the body.

Lymphomas most often develop in the anterior region of the mediastinum and are divided into the following types:

1. Hodgkin's lymphoma. The disease most often affects young (20 to 30) and older people (55-60 and older). Its peculiarity is the presence of giant cells in the affected lymphoid tissue, which are visible under a microscope.
2. Non-Hodgkin's lymphoma. This name includes all types of lymphomas that are not related to lymphogranulomatosis (Hodgkin's lymphoma).

Both lymphogranulomatosis and non-Hodgkin's lymphomas are also divided into types that have different degrees of malignancy and, accordingly, different prognoses.

In the mediastinum zone, nodular sclerosis most often develops - one of the types of Hodgkin's lymphoma - and mainly affects young women.

Lymphadenopathy also often occurs in this area. You can read more about this here.

About 90% of cases of lymphogranulomatosis affect mediastinal lymph nodes, and non-Hodgkin lymphomas - in 50% of cases.

1. Stage I (initial) - lymphoma is located in the area of ​​one lymph node or in one area or organ outside the lymph nodes.
2. Stage II (the disease develops locally) - tumor cells are detected in the area of ​​several lymph nodes located on one side of the diaphragm, or in the area of ​​one lymph node and next to organs or tissues.
3. Stage III (the disease is in an advanced form) - tumor cells are detected in the area of ​​several lymph nodes or in the area of ​​one lymph node and organ located on both sides of the diaphragm. In turn, the third stage is divided into several substages, depending on the severity of the process:

Stage IIIE - tumor cells are detected in the area of ​​​​several lymph nodes above and below the diaphragm, they are also located outside the lymph nodes - in tissues or in one of the nearby organs.

Stage IIIS - tumor cells are detected in the area of ​​​​several lymph nodes above and below the diaphragm and in the spleen.

Stage IIIE + S - tumor cells are detected in the area of ​​​​several lymph nodes above and below the diaphragm, in tissues or in one of the nearby organs and in the spleen.

Stage IV - tumor cells are detected outside the lymph nodes and are located in one or more organs and in the lymph nodes next to them. Also, cancer cells can be detected in many organs and systems located at different distances from the primary cancer site, for example, in the lungs, bones, and liver.

For any lymphoma, a letter (A or B) is added to the stage number:

• A - When the diagnosis was made, the patient did not have symptoms of lymphoma, such as weight loss, fever, and others.
• B - symptoms were present.

Forecast

The likelihood of a complete cure depends on many factors: risk factors, availability of qualified medical treatment (competent, experienced specialists and equipment), stage and form of lymphoma and the general health of the patient.

Risk factors at stages 1 and 2 include:

• the tumor-like formation is more than ten centimeters in size;
• oncology has spread to three or more lymph nodes;
• laboratory analysis determined that red blood cells in the patient’s body have a high sedimentation rate;
• cancer cells are found in one of the organs;
• The patient has specific symptoms of lymphoma.

If none of these risk factors are present, the prognosis is positive.

Risk factors for stages 3 and 4 include:

• age over 40 years;
• lymphoma has moved to stage IV;
• low level of albumin in the blood;
• increased white blood cell count;
• decreased hemoglobin content;
• decreased lymphocyte count;
• male gender.

On average, 90% of patients with non-aggressive stage 1-2 lymphoma who undergo treatment cross the ten-year survival threshold.

If lymphoma has spread widely throughout the body and reached the terminal stage, the chances are significantly reduced: 20-30% of patients survive in the first five years.

Reasons for appearance

At the moment, it is unknown what exact causes provoke the development of lymphoma, but there are a number of risk factors that increase the likelihood of developing the disease:

• People who suffer from or have had certain diseases (benign lymphoblastosis, hepatitis C, systemic lupus erythematosus, rheumatoid arthritis, any other autoimmune diseases) have an increased risk of developing lymphoma. The presence of the Epstein-Barr virus, HIV infection and genetic disorders associated with immunodeficiency (Louis-Barr, Wiskott-Aldrich, Purtillo syndrome) also increases the chances.
• Hereditary factor (if close relatives suffered from diseases of the bone marrow or lymphatic system, this increases the risks).
• Chemotherapy and radiation therapy negatively affect the lymphatic system and the immune system in general, which also increases risks.
• People taking immunosuppressive drugs after an organ transplant are also at risk.
• Work in areas where there is interaction with toxic chemicals, pesticides, carcinogens, and radioactive substances.
• Living in areas with unfavorable environmental conditions.
• The presence of a large amount of animal proteins in the diet.
• Long and regular exposure to the sun, frequent visits to solariums.
• Insufficient diet, which contains a small amount of nutrients and an excess of chemical additives.
• Weak immunity.
• Previously suffered oncological diseases.

Symptoms of the disease

Neoplasms in the lymphatic tissue in the early stages are not accompanied by any symptoms, which complicates diagnosis and increases the likelihood of developing the disease.

Often, formations of this type are discovered by chance: during a medical examination or during the diagnosis of other diseases of the organs of the mediastinal zone using fluorography or radiography.

As the tumor progresses, it negatively affects nearby organs and tissues, and over time, when this impact becomes significant and the cancer cells spread significantly, the first pronounced symptoms will appear, closely related to which organs and areas are affected by the lymphoma.

In the initial stages, some patients experience the following symptoms:

• high degree of fatigue;
• sleep disorders;
• weight loss;
• loss of appetite;
• weakness;
• cough;
• high level of irritability;
• apathetic state;
• weakness of the immune system (manifests itself in frequent infectious diseases that occur in more severe forms);
• increased sweating at night.

Even experienced doctors are not always able to understand what is causing the development of symptoms; there is a high probability of receiving an erroneous diagnosis.

As the disease progresses, previous symptoms worsen and new ones appear, related to which organs and systems are affected by the tumor:

If lymphoma has affected the respiratory system, the following symptoms occur: chest pain when breathing, shortness of breath, frequent coughing accompanied by sputum containing blood, difficulty breathing.

If the formation has affected the esophagus, discomfort is felt during swallowing.

Damage to the spinal cord is accompanied by numbness in the legs and arms, partial paralysis, and difficulty moving and walking.

Compression of the veins and arteries in the mediastinum leads to the development of the following symptoms: frequent and intense pain in the head, pain in the chest, dizziness, arterial hypertension, swelling of the face, bluish skin, shortness of breath.

If the lymphoma is located on the left side of the chest (on the side of the heart), symptoms similar to angina pectoris occur: pain in the chest, tending to radiate to the shoulder, back, arm and other areas, pressing discomfort.

Damage to the laryngeal nerve is accompanied by hoarseness.

If the formation has affected the sympathetic trunk, the following symptoms occur: blepharoptosis, mostly unilateral, the eyeball is sunken, the pupil is dilated, the skin on half the face has an increased temperature, and disruptions in sweat production occur.

The lymph nodes in the groin, armpits and abdominal area are enlarged, but no pain occurs on palpation.

Diagnostics

Diagnostic measures allow specialists to distinguish lymphoma from many other diseases with similar symptoms (for example, tuberculosis, pulmonary sarcoidosis, cystic formations, metastases), make an accurate diagnosis and choose the most effective treatment tactics.

Diagnosis of lymphoma includes:

urine and blood analysis;

palpation of lymph nodes;

performing an ultrasound examination of the chest organs;

computer and magnetic resonance imaging;

chest x-ray;

taking material for biopsy;

lymphoscintigraphy;

examination of cerebrospinal fluid and bone marrow;

endoscopy of the larynx, trachea;

bronchoscopy.

Treatment

The following methods are used in the treatment of lymphomas:

Chemotherapy is the method most often used in the treatment of lymphomas (along with radiation therapy). The patient is prescribed special chemotherapy drugs that have a destructive effect on cancer cells. They are selected individually, based on the type of tumor, health status, age, and additional chronic diseases. A correctly selected drug significantly increases the likelihood of cure, since its effect extends to the entire body and helps eliminate a large number of metastatic foci. Some of the most common chemotherapy drugs are the following: Dacarbazine, Rituximab, Vinblastine.

The patient is also prescribed painkillers. The type of pain medication depends on the intensity of the pain and the stage of the lymphoma:

For mild pain, non-opioid painkillers are used: paracetamol, ibuprofen and others.

If the pain intensifies, weak narcotic drugs are prescribed: tramadol, codeine.

Intense, unbearable pain is relieved with strong opioid drugs containing morphine or other active ingredients. The following drugs are used: Bupranal, Dipidolor, Durogesic.

Prevention

Fully following certain tips will not provide a 100% chance of avoiding mediastinal lymphoma, but will significantly reduce the risks.

To reduce the likelihood of developing lymphoma, you should consider the following recommendations:

• avoid excess ultraviolet radiation (reduce the frequency of visits to the solarium, sunbathe moderately);
• eat properly and nutritiously, include more vegetables and fruits in your diet;
• workers in hazardous industries must carefully follow all safety regulations;
• if possible, you should choose areas with the best environmental conditions for living; It will also be useful, if this is not possible, to periodically travel to areas with a good environmental situation for recreation;
• undergo regular medical examinations and monitor your health;
• promptly treat all inflammatory processes in the body;
• strengthen the immune system;
• avoid unprotected sexual contact, use condoms (prevention of HIV, hepatitis C and other diseases that increase the likelihood of developing lymphoma);
• lead an active lifestyle, spend more time in the fresh air.

If mediastinal lymphoma is detected in the early stages of development, the chances of curing it completely are high, so it is necessary to go to the hospital at the first symptoms and follow all the recommendations of a specialist.

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Attention! The materials on the site are published for informational purposes only and under no circumstances can be considered a substitute for medical consultation with a specialist in a medical institution. The site administration is not responsible for the results of using the posted information. For questions of diagnosis and treatment, as well as prescribing medications and determining their dosage regimen, we recommend that you consult a doctor. Remember: self-medication is dangerous!

Mediastinal lymphoma

The mediastinum is the anatomical space of the middle part of the thoracic cavity. Various sources offer different options for dividing this department into areas. The most common classification involves the division into anterior, middle and posterior zones.

Primary lymphomas of this type are predominantly sluggish (indolent) neoplasms of the non-Hodgkin type, which represent either degeneration of lymph node tissue or dysplasia of lymphocytes in internal organs. The most common type is B-cell lymphoma.

In rare cases, aggressive forms of lymphomas (T-cell) are diagnosed, which grow into nearby anatomical structures - trachea, bronchi, pleural cavity, esophagus.

• All information on the site is for informational purposes only and is NOT a guide to action!
• Only a DOCTOR can give you an ACCURATE DIAGNOSIS!
• We kindly ask you NOT to self-medicate, but to make an appointment with a specialist!
• Health to you and your loved ones! Do not give up

Let's take a closer look at what it is - how these tumors manifest themselves, how they are detected, and what methods are used to treat them.

Causes

The true causes of primary mediastinal lymphomas (as well as lymphogenous tumors in general) are not known to medicine. Doctors note only a trend toward a general increase in the number of cases of non-Hodgkin lymphoma diagnosed throughout the world.

Presumably, this is due to the poor nutrition of modern people, who consume an increasing amount of foods with chemical additives (in particular, vegetables with pesticides). The general deterioration of the environmental situation in developed countries of the world also plays a negative role.

Other possible risk factors for malignant neoplasms of the lymphatic system include:

• exposure to radiation (including radiation therapy for other oncological pathologies);
• contact with carcinogenic substances in chemical production;
• working with pesticides in agriculture;
• the presence in the body of aggressive viruses that affect the state of the immune system (for example, the immunodeficiency virus or the Epstein-Barr virus);
• the presence of autoimmune diseases (congenital and acquired);
• elderly age.

In childhood, the likelihood of lymphoma occurring is relatively low - only a twentieth of all diagnosed cases occur before the age of 30, while males are more likely to get sick.

Symptoms

Lymphatic tumors of the mediastinum can be primary and secondary. In the first variant, neoplasms arise initially in the area under consideration, in the second they are metastases formed as a result of the spread of a malignant process that began in another part of the body.

The nature of the lymphogenous spread of secondary lesions is associated with the characteristics of lymph outflow from the affected organ. Secondary enlargement of the nodes of the lymphatic system of the mediastinum is most often caused by lung (bronchial) cancer: this disease gives metastases in 80% of cases. A less common source of secondary lymph node involvement is malignant tumors of the digestive tract.

Symptoms of mediastinal lymphomas depend on the location of the lesion.

If tumors have arisen in the lung area and have already spread to the respiratory organs, the symptoms characteristic of lung cancer and sarcoma come first:

• dyspnea;
• cough;
• secretion of sputum mixed with blood;
• pain when breathing in the sternum.

When the trachea or esophagus is affected, problems with swallowing food (dysphagia) are often observed. When the tumor damages the structures of the spine and involves the nerve endings of the spinal cord in the process, radicular syndromes characteristic of vertebral hernias may occur - numbness of the limbs, problems with motor activity, problems with control of the pelvic organs, paresis and paralysis.

In addition, symptoms common to all cancer diseases develop:

• weight loss due to decreased appetite;
• night sweats;
• apathy, irritability;
• weakness, deterioration in performance;
• periodic increase in temperature;
• decreased immune status.

The last point leads to infectious diseases - both bacterial, viral and fungal. Due to the lack of adequate body protection, such diseases are very difficult for people with mediastinal lymphoma.

Diagnostics

Detection of non-Hodgkin lymphomas in general (and mediastinal lymphomas in particular) is a rather labor-intensive and often lengthy process, which includes the use of various diagnostic methods and techniques.

Among the diagnostic procedures practiced by oncologists:

• palpation of lymph nodes;
• external examination of the patient;
• X-ray of the lungs;
• Ultrasound of internal organs;
• CT scan;
• endoscopic methods for examining the larynx and bronchi;
• biopsy of the tumor node and further histological and immunological examination of the tissue in the laboratory;
• lumbar puncture (examination of cerebrospinal fluid for the presence of cancer cells);
• bone marrow biopsy (like all lymphomas, mediastinal lymphoma carries a potential risk of developing leukemia);
• detailed blood test.

The most revealing is the histological analysis of the biopsy specimen. Examination of a tumor sample allows us to determine the degree of malignancy of the disease, the stage of its development and prescribe the most effective therapy.

Video: Lymphoma in detail

Treatment

The main method in the treatment of primary mediastinal lymphomas is systemic chemotherapy. The entire body is exposed to medicinal effects, since the lymphatic system and lymphocytes are found in almost all tissues and organs.

There are a number of chemotherapy protocols for different types of non-Hodgkin's lymphoma. Different drugs are used to treat B-cell and T-cell diseases. Non-aggressive and indolent types of mediastinal lymphomas are treated with standard courses of cytostatics.

Treatment with MabThera (Rituximab) gives good results, sometimes in combination with standard cytostatics. This medicine belongs to the group of monoclonal antibodies: it provides a targeted effect on tumor cells and has minimal side effects.

Radiation therapy and surgical treatment are auxiliary. Tumors are exposed to radiation or excised only in cases where there are direct medical indications for this.

Photos of Hodgkin's lymphoma can be seen here.

Prognosis for mediastinal lymphoma

The prognosis of treatment and survival of patients with mediastinal lymphatic tumors depends on many factors:

• age of patients;
• degree of damage to the immune system;
• localization of the tumor;
• the degree of damage to the nodes of the lymphatic system;
• stages of disease development.

Non-aggressive forms of non-Hodgkin lymphoma at the first stage are relatively successfully treated. The survival rate of patients over a 10-year period is about 90%. If the disease has reached the stage of spreading through the circulatory system or has penetrated the bone marrow, the prognosis worsens: only 20-25% of patients survive for several years.

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The information on the site is provided solely for popular informational purposes, does not claim to be reference or medical accuracy, and is not a guide to action.

Do not self-medicate. Consult your healthcare provider.

Mediastinal tumors can be benign or malignant. These include lymphomas - neoplasms that affect lymphoid tissue. Most often, mediastinal lymphoma occurs in young people under 40 years of age.

Description and statistics

Many people are not familiar with the term "mediastinum". This is the area located between the spine and the chest, where important human organs are located - the lungs, heart, blood and lymphatic vessels, and nerve endings.

Benign mediastinal lymphoma is an intermediate form between simple and malignant lymphoma. A simple one is a formation consisting of lymph cells that occurs against the background of chronic inflammatory processes in internal organs or during the regeneration of lymphoid tissue.

Benign lymphoma develops most often in the lymph nodes - cervical, axillary, etc. It has a nodular structure, dense to the touch, which is characterized by extremely slow growth and development. Its origin is associated with chronic inflammatory processes in the lymphatic system.

Approximately 25% of benign lymphomas eventually transform into malignant ones, so they require mandatory treatment. An atypical process is formed against the background of cell mutation or becomes the result of metastasis of tumors from other parts of the body.

Malignant lymphoma is characterized by rapid progression of the disease, in which all organs of the mediastinum and mediastinal lymph nodes can suffer - intrapulmonary, aortic, etc. Untreated pathology is dangerous because it grows into the main nerves and vessels, damages the digestive and respiratory systems, which in turn is fraught with many complications.

According to statistics, mediastinal lymphoma occurs in 3% of cases among other types of malignant changes in lymphoid tissue. Mostly the development of the disease is observed at the age of 20-40 years, regardless of the person’s gender. Cases of mediastinal lymphoma have also been reported in children and people over 70 years of age.

ICD-10 code: C81 - C96 Malignant neoplasms of lymphoid, hematopoietic and other related tissues.

Causes and risk group

Factors contributing to the development of lymphoma affecting the mediastinal organs are not fully understood. But oncologists were able to identify a number of reasons in the general group that may contribute to the occurrence of a malignant process:

• unbalanced diet with products containing a large number of unnatural ingredients;
• prolonged contact with carcinogenic substances, for example, in chemical production conditions;
• exposure to pesticides entering plants from mineral fertilizers;
• influence of ionizing radiation;
• treatment of cancer in the past using radiation therapy;
• congenital or acquired immunodeficiency;
• untreated chronic infectious and inflammatory processes of internal organs.

In recent years, there has been a worldwide trend towards an increase in lymphomas in oncology. Presumably, the risk group for the disease includes people with weak immunity and frequent viral infections.

Also, a hereditary factor cannot be ruled out - pathology is more common in people whose blood relatives have already encountered lesions of the blood and lymph, both benign and malignant.

We also need to pay attention to the external influence of the environment that surrounds us. The polluted atmosphere of megacities, poor quality of food and drinking water, stress - all this negatively affects the state of the immune defense of a modern person, and therefore malfunctions can occur in it, sometimes leading to malignant processes in the body.

Symptoms

Benign lymphoma does not cause any signs of disease other than enlarged lymph nodes. The same can be said about malignant lesions of lymphoid tissue, since it can be diagnosed at stages I and II only by chance, for example, by performing routine fluorography or radiography of the bronchi and lungs for prophylactic purposes or for the treatment of a certain disease.

As the lymphoma progresses, it begins to exert abnormal pressure or grow from the primary site into adjacent anatomical structures. All this negatively affects the functioning of the affected organs. The symptoms of the disease directly depend on which of them has malignancy of the lymphoid tissue.

In case of damage to the respiratory system - bronchi and lungs - the signs of lymphoma will be as follows:

• dyspnea;
• chest pain;
• cough with sputum containing blood streaks.

If the tumor process puts pathological pressure on the spinal cord or grows into its structure, the person will experience the following symptoms:

• numbness in the upper and lower extremities;
• paresis and paralysis;
• persistent motor activity disorders.

Lymphoma, which affects the tissues of the trachea and esophagus, interferes with proper nutrition, causing problems with the act of swallowing.

If the tumor process compresses or spreads to the venous trunks of the mediastinum, a person experiences the following symptoms:

• dizziness;
• migraine;
• chest pain;
• bulging of large veins in the neck;
• swelling and bluish complexion;
• increased blood pressure.

If the lymphoma is located in the left part of the mediastinum, the clinical picture of the disease will be similar to angina pectoris.

If the sympathetic fibers of the nervous system are involved in the tumor process, then the person develops symptoms of vegetative-vascular dystonia and ophthalmological problems arise, such as ptosis or drooping of the eyelid, retraction of the eyeball, and unnatural dilation of the pupil.

When the lymphoma is in close proximity to the laryngeal nerve, the patient experiences increased hoarseness.

In the later stages of the oncological process, all mediastinal lymphomas are characterized by common symptoms:

• constant low-grade body temperature;
• sweating, worse at night;
• weakness, apathy;
• lack of appetite and weight loss;
• immunodeficiency and associated frequent infections with a long and complex course.

It should also be noted dermatological manifestations characteristic of certain types of lymphomas. With large neoplasms, a person may experience itching, rashes on the skin, mainly in the affected area, and the development of plaques and ulcers. Additionally, there is an increase in lymph nodes (axillary, submandibular, cervical and inguinal), which often grow to the size of a chicken egg, but do not cause pain on palpation - evidence that the nature of their growth was not infection or inflammation, but atypical changes.

Stages

We invite you to find out how malignant mediastinal lymphoma develops according to the international Ann Arbor classification in the following table.

Stages	Description
I	The tumor is localized in one lymph node or in a limited area of ​​a certain organ, in its lymphoid tissue. There are no symptoms of the disease.
II	Malignant cells are detected simultaneously in several lymph nodes located on one part of the diaphragm or in a single lymph node, but in close proximity to adjacent anatomical structures - tissues and neighboring organs. Signs of pathology are absent or weakly expressed.
III	The tumor process has started, atypical cells are diagnosed in multiple lymph nodes located on both sides of the diaphragm, simultaneously affecting nearby tissues and organs. Clinical signs of the pathology are pronounced, the disease becomes progressive.
IV	Malignant cells are detected not only in the mediastinum area, but also in distant organs and lymph nodes. The pathology is considered incurable; only 2% of patients manage to cross the 5-year survival threshold.

Additionally, the stages of mediastinal lymphoma, according to the Ann Arbor system, are marked with the letters “A” and “B”:

• “A” - the patient does not have symptoms such as weight loss, hyperthermia, weakness, etc.;
• “B” - all of the above signs of pathology are present.

Types, types, forms

Mediastinal lymphomas can be primary or secondary.

Primary neoplasms are located closer to the chest and are divided into two types - Hodgkin's and non-Hodgkin's.

Secondary lymphomas are the result of the spread of atypical cellular structures from other cancer foci. Getting into the mediastinum area through the lymph and blood flow, malignant elements affect its organs, forming new tumor processes. The prognosis for secondary formations is sharply negative.

Hodgkin's lymphoma is also called lymphogranulomatosis. They begin development from B and T lymphocytes. Pathologies in most cases are characterized by an asymptomatic course. As the disease progresses, clinical manifestations of a general type characteristic of cancer intoxication are noted - these include weakness, weight loss, hyperthermia, and night sweats. Hodgkin's lymphomas are more common in young people under 30 years of age.

Non-Hodgkin's lesions, on the other hand, are usually diagnosed in older individuals—predominantly in elderly patients. Such lymphomas can be classified into the following forms:

• Lymphosarcoma. In turn, it is divided into diffuse and nodular. In the first case, the tumor has a pronounced aggressive nature, no growth boundaries, and therefore quickly spreads to adjacent structures; in the second, the tumor forms in the form of a follicular focus, causing lesions in the mediastinal organs.
• Reticulosarcoma. The origin of this lymphoma is not fully understood, some oncologists believe that it develops from reticular cells of blood vessels, others - it is a consequence of lymphosarcoma, in any case, the disease is diagnosed extremely rarely, the cancer focus has blurred features and a high degree of aggression.
• Giant cell (large cell) lymphoma. Visually it looks like numerous nodular changes connected into a common tumor. The pathology quickly grows and metastasizes, developing mainly in the tissues of the mediastinal parenchyma, mainly in the anterior part.

Non-Hodgkin's tumors are characterized by rapid progression, damage to adjacent organs and tissues and disruption of their functioning.

Diagnostics

If there is a suspicion of the development of malignant changes in the mediastinum, it is necessary to consult a doctor as soon as possible and undergo a comprehensive examination.

The standard list of laboratory and instrumental procedures includes the following activities:

• blood tests - allow you to determine the presence of inflammatory changes in the body and criteria indicating disturbances in its functioning. Attention is also drawn to such a marker as lactate dehydrogenase; with malignant lesions of lymphoid tissue, its levels increase sharply;
• biopsy of a tumor, lymph node - allows you to identify cells that have undergone atypia in the body, thereby confirming the alleged diagnosis;
• spinal cord puncture - studying the composition of the cerebrospinal fluid helps determine the presence of cancer in it;
• Ultrasound and radiography are techniques that quickly and painlessly detect the presence of tumors and metastases in the mediastinal organs - in the stomach, spleen, lungs, etc.;
• endoscopy - this type of diagnosis allows you to obtain information about the condition of the larynx and bronchi;
• MRI and CT - both methods assess the prevalence of lymphoma in the body, identifying the exact localization of regional and distant metastases much more effectively than x-rays.

Biopsy is considered the most revealing method among the above. It makes it possible to determine the structural structure of the neoplasm and the degree of its malignancy. Despite this, diagnosis must certainly be comprehensive.

Treatment

For all persons faced with mediastinal lymphoma, therapeutic tactics are selected individually. This disease can be treated using methods that we will list below.

Chemotherapy. The main way to combat damage to lymphoid tissue is based on taking cytostatic drugs that destroy atypical cells. It is noteworthy in that the medications used in the treatment process affect the entire body, that is, theoretically, those malignant foci that were not identified during diagnosis can also undergo decay.

For mediastinal lymphomas, cytostatics such as Vinblastine, Dacarbazine and Doxorubicin are more often used. Often, a monoclonal drug, Rituximab, is added to the complex of medications listed above. This chemotherapy regimen, according to oncologists, has maximum success.

Unfortunately, in addition to its advantages, this technique also has its disadvantages, but without its use, a rapid fatal outcome in progressive lymphoma is almost inevitable. At the last terminal stage of the disease, a person’s life is supported exclusively with the help of chemotherapeutic agents.

Radiation therapy. The technique is used if there is information about the exact location where the tumor process is located in the mediastinum. It is usually not prescribed independently; as a rule, it is combined with the use of cytostatic agents.

In recent years, in the fight against lymphomas, including those localized in the mediastinal region, radiation therapy has been used more often due to the advantages of this method. The best results are achieved with the simultaneous use of chemotherapy drugs and ionizing rays that precisely target malignant tissue.

The mechanism of action of this approach is simple - a cytostatic agent, penetrating into the tumor site, blocks the functionality of the blood vessels feeding the tumor, which causes its further disintegration, and radiation exposure destroys the structure of cancer cells faster.

As an independent method, irradiation is usually used in the later stages of lymphoma for palliative purposes, in order to alleviate its symptoms and prolong the patient’s life.

Surgical treatment. In practice, it is used extremely rarely, mainly only at the very beginning of the pathology.

In the case of widespread spread of lymphoma and involvement of vital organs (main vessels, nerves, lungs, etc.) in the malignant process, during surgery, irreversible damage to their structure is possible, and therefore surgical methods are practically not prescribed in the later stages.

In addition to treating the underlying disease, patients need adequate pain relief therapy aimed at eliminating the clinical symptoms of the disease. The type of analgesic will depend on the severity of the patient's pain:

• mild discomfort - non-opioid drugs: Ibuprofen, Paracetamol, etc.;
• severe pain - mild narcotics: Codeine, Tramadol, etc.;
• unbearable, extremely intense sensations - opioid medications containing morphine and other ingredients as the active ingredient: Dipidolor, Bupranal, etc.

Traditional treatment. Alternative medicine methods can never become an alternative to official treatment - every adult should be clearly aware of this. By trying to amend treatment regimens prescribed by an oncologist, or by independently applying folk recipes in practice in the form of medicinal herbs and animal products, such as flaxseed, without consulting a doctor, the patient exposes himself to unjustified risk with his own hands, as in the old days.

Bone marrow/cell transplant

Bone marrow is represented by spongy tissue located in large bones - femurs, ribs, etc. It contains stem cells that produce blood cells. In patients with lymphomas, leukemia and immune pathologies, stem cells synthesize too many damaged and immature elements.

Defective cells fill the general bloodstream, displacing healthy structures. Chemotherapy used to treat lymphomas destroys not only abnormal blood cells, but also normal blood cells and bone marrow structure. Therefore, doctors often recommend its transplantation - replacing damaged elements with healthy biomaterial. Of course, the method cannot give a 100% guarantee that a person will be completely cured of the disease, but the likelihood of stable remission increases significantly.

Transplantation may be as follows:

• autologous - biomaterial is taken from the patient himself;
• syngeneic - the bone marrow of a twin or brother and sister is used, which has high criteria for similarity in structure;
• allogeneic - transplantation of biomaterial from a donor.

The transplantation process consists of the following steps:

1. Preparatory.
2. Bone marrow removal.
3. Processing of biomaterial.
4. Transfer.
5. Engraftment.

The first stage lasts up to 10 days. At this point, intensive courses of chemotherapy are carried out, and anti-inflammatory and antihistamines are prescribed to avoid acute immunodeficiency.

Own bone marrow, if we are talking about an autologous transplant, is removed from the body by condensation. This procedure is more complex than chemotherapy; against its background, the patient receives enormous doses of toxic medications in combination with radiation therapy. Patients are in a stable and serious condition, experiencing massive pressure on the functioning of the entire body. In case of failure, the situation is considered irreversible and the person dies, despite the original purpose of the event.

The donor also goes through a preparatory stage. Its genetic compatibility with the recipient is studied, and contraindications to the procedure are excluded.

Actually, the process of bone marrow collection itself is safe and practically does not cause pain. Modern methods for obtaining biomaterial are based on the mass expulsion of stem cells into the bloodstream through the use of certain drugs, from where they are removed from the body and processed. This process is called apheresis.

Less commonly, bone marrow is removed from the donor's long bones. In this case, hospitalization in a hospital for a day and collection of biomaterial under anesthesia with a special syringe are indicated. The volume of bone material removed does not exceed 10-20 ml. In the future, it is restored within 1-2 months, that is, theoretically, the entire procedure does not pose any danger.

Bone marrow transplantation into the recipient's body takes the form of a traditional injection of biomaterial into the systemic bloodstream. Once there, the cells begin to spread throughout the body, settling in the bone tissue and beginning active engraftment.

After positive dynamics occur and the injected substance begins to take root and carry out its functions, the person’s well-being will gradually improve - the temperature will disappear, the symptoms of inflammation and enlarged lymph nodes will disappear, which is most typical for lymphomas. At this stage, doctors constantly monitor the patient, trying in every possible way to prevent possible complications.

Nowadays, instead of bone marrow, patients are often transplanted with stem cells if the donor is genetically compatible with the recipient. To do this, over 4 days the donor is given medications that enhance the release of stem cells into the general circulation. This preparation does not cause side effects, only sometimes cold-like symptoms and slight hyperthermia may occur. After the end of the primary stage, the biomaterial is collected: a special device is inserted into a vein of one arm, collecting blood and filtering exclusively stem cells, after which the blood enters the body again, but through the other arm. All manipulations last several hours, do not require pain relief and are well tolerated by donors. The stem cells are then processed and injected into the recipient according to the principle described above.

Israel is deservedly considered a country specializing in donor organ transplantation, but in Russia these manipulations are carried out at the proper level according to exactly the same standards. Donors who are ready to donate their bone marrow are entered into the registers of the all-Russian database. Patients who require a transplant are also recorded in the data of the clinic where they received or are undergoing treatment, after which their compatibility with potential donors is analyzed.

In Israel, the cost of a transplant is lower than in European countries - from 100 thousand dollars. In Germany, the same figures are announced, but in euro equivalent.

Where can I go?

• Russian Scientific Center for Surgery named after. B.V. Petrovsky, Moscow.
• Clinic "Charite", Berlin, Germany.
• Clinic "Assuta", Tel Aviv, Israel.

Recovery process after treatment

Monitoring a person who has undergone complex therapy for lymphoma is a mandatory component of treatment. It allows you to timely diagnose complications that often arise during the fight against the main cancer process, and identify tumor relapses. In addition, the goals of rehabilitation are considered to be the solution of social, spiritual and physical problems of a person. On average, the recovery process lasts at least 4 weeks.

The main focus at this stage is nutrition. A person who has undergone a course of intensive chemotherapy and radiation needs individual dietary adjustments. This allows him to quickly restore lost functions and strength of the body.

The menu may include fresh fruits and vegetables, juices, vegetarian soups and side dishes, cereals and legumes, fish and seafood, poultry and rabbit meat, and fermented milk drinks. Proper drinking regimen is also important - at least 2 liters of water per day to relieve intoxication and cleanse the body of poisons and toxins.

Alcohol, fried and fatty foods, canned food and pickles, sugar, and baked goods made from white flour are prohibited. Salt is allowed, but of course in small quantities.

In addition to diet, during the rehabilitation process a person needs moderate physical activity, certain physical procedures, and walks in the fresh air. Additionally, you may need the help of a psychologist, and the support of relatives is required.

Course and treatment of the disease in children, pregnant and lactating women, the elderly

Children. In childhood, according to statistics, mediastinal lymphomas are rare. The development of pathology can be facilitated by congenital factors, for example, immunodeficiency received from the mother, viral infections, etc. The clinical manifestations of the disease are practically no different from adults - the child develops a cough, shortness of breath, enlarged lymph nodes, hyperthermia, etc.

Due to the fact that lymphogenous lesions are rare in children, early signs of the disease may not be detected in time and the small patient ends up with a hematologist or oncologist with an advanced form of the cancer process. In this case, it will be difficult to talk about a favorable prognosis for him, despite the fact that the younger the patient, the better the expected outcome of the disease.

After confirming the diagnosis, doctors select individual treatment tactics. As a rule, it begins with a combination of chemotherapy and radiation courses, and if the expected effect is not achieved, the child may undergo surgery and a bone marrow transplant may be recommended.

Pregnant women. Pathology is also rare in expectant mothers. Mediastinal lymphoma during pregnancy is manifested by the growth of lymph nodes, an unreasonable increase in temperature, profuse sweating at night and symptoms of compression of internal organs in the chest. The causes of the disease are not fully understood, but in women who are pregnant, hormonal imbalance is primarily considered.

After confirming the diagnosis, an individual course of treatment is selected for the patient and the issue of prolonging the pregnancy is decided. If we are talking about the first trimester, doctors recommend having an abortion and immediately starting to fight the underlying disease. As a rule, mono- and polychemotherapy methods are used, which are supplemented by irradiation of the lesions.

If it is decided to continue the pregnancy, it is not advisable to treat lymphoma using these methods. When the condition allows you to wait, that is, the oncological process is sluggish, therapy is postponed until the postpartum period. In other cases, treatment tactics are selected individually.

Nursing. Mediastinal lymphomas in nursing mothers can occur against the background of weakened immunity, which often haunts a woman after the birth of a child. Unfortunately, many patients notice the pathological process with a significant delay, since for a long time they remain absorbed in concerns about the baby, and not about their own health. Advanced forms of the disease are more difficult to cure; in order to achieve stable remission, you will have to put in more effort and time.

Once lymphoma is diagnosed, a treatment plan is selected. Typically, intensive chemotherapy and radiation are used to combat this pathology - methods that are incompatible with continued breastfeeding. The prognosis will depend on the stage of the oncological process, spread and type of tumor.

Elderly. Lymphomas become more common with age. Many patients do not pay attention to the onset of the disease, attributing the symptoms that arise to existing and concomitant pathologies. Most often, weak immunity, an impressive history of harmful addictions, many years of work in hazardous industries, poor nutrition and chronic infections are to blame for the development of the disease.

The clinical picture of mediastinal lymphoma depends on which organ is affected by the oncological process. As it progresses, the general symptoms of the disease increase, and the person’s condition worsens sharply. In old age, many treatment methods are associated with various contraindications and restrictions, so treatment tactics are selected by a specialist on an individual basis. The prognosis depends on the timeliness of the diagnosis and the initiation of treatment measures in relation to it, but with age the chances of remission only worsen.

Treatment of mediastinal lymphoma in Russia, Israel and Germany

We invite you to find out how the fight against mediastinal lymphomas is carried out in different countries.

Treatment in Russia

After contacting a specialist, a comprehensive examination is carried out, including laboratory tests, ultrasound, MRI and biopsy to study the histological structure of the tumor. If the diagnosis is confirmed, Russian specialists select the necessary treatment tactics, depending on the size of the lymphoma and the patient’s health condition.

Basic methods of combating the disease:

• surgical intervention aimed at removing the affected lymph nodes;
• radiotherapy - used after surgical treatment;
• chemotherapy using high doses of cytostatics;
• bone marrow transplantation.

By combining the methods listed above, doctors manage to block the spread of lymphoma to the mediastinal organs, achieve stable remission and prolong a person’s life. It is important to start treatment on time.

The cost of fighting the disease in Moscow and other Russian cities depends on the type and location of the tumor process, the chosen treatment tactics and the characteristics of rehabilitation. On average, the amount ranges from 150 thousand rubles and above.

• Russian Oncology Research Center (RORC) named after. N. N. Blokhina, Moscow. Outstanding healthcare professionals work here. The clinic treats adult and pediatric oncology.
• The center provides the full range of necessary assistance to persons with pre-tumor and malignant lesions of internal organs and systems.

Vladlena, 34 years old.“In 2016, I was diagnosed with lymphoma, I contacted the Russian Cancer Research Center named after. Blokhina. I had to endure so much fear while the diagnosis was going on, the first courses of chemotherapy, and I was oppressed by despair. Now everything is behind me and I understand that I can live long and happily if I follow the doctor’s recommendations. Take care of yourself".

Treatment in Germany

In German oncology centers, experienced oncologists are successfully fighting mediastinal lymphomas. The choice of treatment protocol depends on the type of tumor spread. For local lesions, lymphogranulomatosis, local radiation exposure is recommended. In case of an isolated process in the mediastinal lymph nodes - their surgical excision with further radiotherapy. For common cancer processes, polychemotherapy methods are used.

If mediastinal lymphosarcoma is diagnosed, German doctors prefer a combination of irradiation and the administration of cytostatic agents. Most thoracic surgeons and oncologists in the country believe that lymphomas of this localization should necessarily be subjected to surgical excision. In the case where the tumor process is operable, intervention can be carried out already at the diagnostic stage in the form of a total biopsy of the suspicious lesion. In difficult cases, a bone marrow transplant is performed.

The cost of treatment depends on the characteristics of mediastinal lymphoma and the list of necessary therapeutic measures. On average, the total amount is from 70 thousand euros.

Which clinics in Germany can I go to?

• A multidisciplinary medical institution that provides specialized care to people facing cancer. The clinic also performs stem cell and bone marrow transplantation operations.
• University Hospital of Freiburg. Oncologists of this center use advanced achievements of medicine and science, skillfully applying them in the fight against various oncological diseases, including those of a lymphogenous nature.

Let's look at the reviews of the listed clinics.

Anna, 56 years old. “The doctors diagnosed lymphoma, I started coughing and getting sick all the time, I had to quit my job. I applied for treatment to Germany, to the Charite clinic. I’m satisfied with the attitude of the doctors and the assistance provided, everything went well.”

Martha, 45 years old. “In a clinic in Freiburg, my son had mediastinal lymphoma removed and received chemotherapy. “I am very grateful to the doctors for the work done and the result obtained.”

Treatment of mediastinal lymphoma in Israel

The methods used in Israeli clinics differ from traditional ones in their minimally invasiveness, the shortest rehabilitation period, rapid recovery and return to a full life. The main ways to combat the disease:

• chemotherapy - is implemented using the latest generation of cytostatic agents that have a minimum of adverse reactions and high antitumor efficacy;
• targeted therapy - widely used in Israel, based on the use of agents that specifically destroy malignant cells without harming healthy tissues;
• CAR-T therapy is an innovative approach that can lead to stable remission in more than 50% of patients. For treatment, the immune cells of the person themselves are used, processed in the laboratory using special technology and capable of destroying cancer cells in the body;
• radioimmunotherapy is a promising method of combating oncology, working at the cellular level in relation to atypical tissues;
• radiation therapy - all Israeli clinics are equipped with powerful modern technologies that allow irradiation of tumor processes using remote, stereotactic, modulated and other programs;
• Bone marrow transplantation is carried out after high-dose chemotherapy, radiation and in the later stages of the disease. In this country, all types of internal organ transplantation are available, including from an unrelated donor.

Before starting treatment, doctors gather a consultation and discuss with the patient the likely risks and complications that may arise with each of the above options for therapeutic intervention. The cost of treatment for mediastinal lymphoma depends on the amount of assistance required; for each patient it is determined individually. On average, prices for therapy can range from 50 thousand dollars; if a bone marrow transplant is needed, the amount increases several times.

Which clinics can I go to?

• The best doctors work here, widely known for their work achievements outside the country.
• Rambam Medical Center, Haifa. A multidisciplinary medical institution that provides a full range of care to people with benign and malignant diseases.

Let's look at the reviews of the listed clinics.

Maya, 47 years old. “About 7 years ago, I began to experience growth of lymph nodes in my neck; I didn’t attach any importance to it for a long time until the tumor became visible in photographs. After going to the doctor, they diagnosed lymphoma, but for a long time they could not decide whether it was benign or malignant. Biopsies at two different sites gave dissimilar results. Tired of uncertainty, I turned to Israel, to the Ichilov hospital. Here, not only was the type of lymphoma determined within a few days - it turned out to be lymphoblastic, but also successful treatment was carried out. Thanks to the doctors."

Marina, 35 years old. “For some time, my sister complained of back pain; an x-ray revealed a tumor, which, as it turned out later, was lymphoma of the posterior part of the mediastinum. A description of the pathology was found on the Internet, and there they found the Rambam clinic with good reviews about the treatment of this disease. We called, went, the therapy turned out to be effective, and returned home with stable remission.”

Complications and metastases

Complications arising from lymphomas worsen the quality of life and prognosis for a person, often leading to his death.

The main consequence of the disease is secondary tumors, or metastases. Given the aggressiveness of most lymphomas, atypical cells spread throughout the body quickly, affecting adjacent and distant anatomical structures. Secondary tumors worsen the course of the pathology, complicate the treatment process and generally adversely affect its prognosis.

In addition to metastases, the following complications may occur:

• leukemia and solid neoplasms - the consequences that occur after irradiation of the body reduce the survival prognosis by 20%;
• disorders of the heart and blood vessels: cardiomyopathy, arrhythmia, hypertension, etc.;
• thyroid disorders;
• dysfunction of the reproductive system: infertility in men and women, erectile problems in the stronger sex.

Relapses

Patients who consult a doctor with mediastinal lymphoma on time have a favorable chance of establishing long-term remission and recovery. In such cases, you can expect to overcome the 5-year survival threshold.

Activation of the oncological process a second time is called a relapse; unfortunately, it is observed in many patients with this diagnosis - in almost 50% of cases. In this situation, chemotherapy is again recommended; if there is no effect, bone marrow transplantation is recommended.

Getting a disability

Any cancer is grounds for disability. For mediastinal lymphomas, the following types and conditions of activity are contraindicated:

• moderate and heavy work;
• work associated with intense neuro-emotional stress;
• unfavorable external meteorological factors;
• interaction with industrial toxic substances and ionizing radiation.

Indications for contacting the ITU Bureau (medical and social examination):

• the need to employ patients with low-grade lymphomas;
• the presence of lymphomas of moderate and high malignancy;
• progressive oncological process;
• development of complications.

A minimum of diagnostic measures should confirm the stage of the disease and diagnosis. It includes standard laboratory and instrumental studies, as well as the conclusion of the attending physician.

Criteria for assigning disability:

• Group III - is established for persons with low-grade lymphomas that do not require treatment, in the absence of complications and remission for more than 2 years;
• Group II - prescribed to patients with lymphomas of moderate malignancy at stages I and II in the case of initial examination and stage III in stable remission;
• Group I - is established for persons with lymphomas of moderate and high malignancy of stages III and IV in the absence of positive dynamics on the treatment, progression of the disease with impaired self-care capabilities and an unfavorable prognosis.

After 2 years, most patients must undergo re-examination, after which the group can be replaced with a light one or canceled altogether.

Forecast for different stages and forms

It is impossible to achieve complete recovery even with benign mediastinal lymphoma. Treatment can lead to stable remission and subsidence of the clinical signs of the pathology, but after a while the disease makes itself felt again, so the patient must be registered at the dispensary and visit a specialist on time.

The expected prognosis for malignant mediastinal lymphoma is based on the location of the tumor and the degree of its spread, the stage of the disease and the patient’s condition. Consider the overall 5-year survival rates in the following table.

Diet

Nutrition for mediastinal lymphoma is aimed at strengthening the immune system and normalizing metabolic processes and vital functions of the body. The main recommendation is a diet rich in protein, calories and vitamins that have a positive effect on hematopoiesis and hemoglobin titer, which is necessary to adequately support the defense system in the fight against cancer.

Each patient is selected an individual diet by the attending physician. There can be no talk of any amateur performance. There are lists of permitted and prohibited products for lymphomas and other cancers, but they should also be monitored by a specialist. In this case, it is recommended to eat 6 meals a day, with a small amount of dishes, since people who have undergone courses of radiation and chemotherapy suffer from problems with appetite and simply cannot eat large portions at a time.

At the same time, there is no need to limit the diet of a person with lymphoma. Considering the patient’s aversion to many foods, it is important not to insist on eating prepared foods, but to offer the patient the food that he really wants or likes, of course, with the permission of the attending physician.

It is important to follow dietary principles during the period of established remission - they will help prevent relapse of the pathology.

Prevention

There are no measures that could 100% prevent the development of mediastinal lymphoma. But there are recommendations that can reduce the risk of developing this pathology:

• avoid direct sunlight;
• eat properly and balanced;
• follow safety rules when working in hazardous industries;
• choose environmentally friendly places for permanent residence and recreation;
• undergo regular preventive medical examinations and be more attentive to your own health;
• comprehensively strengthen the immune system;
• give up promiscuity - preventing infection with HIV, hepatitis and other diseases is a specific measure to prevent lymphoma.

Treatment of lymphoma with folk remedies is excluded. If the disease is diagnosed at the initial stages of the oncological process, the patient has a good chance of coping with it and achieving stable remission; the main thing is to follow the doctor’s recommendations in everything.

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Valery Zolotov

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The mediastinum is located in the middle part of the chest. In this anatomical space its anterior, middle and posterior zones fit.

Disease occurs in this area due to uncontrolled cell division. Neoplasms of this type are quite rare, but they are insidious. These need to be treated immediately.

They arise with the primary focus. If treatment is not started, metastasis will occur in other organs. Like everything else, this lymphoma has a malicious quality: it spreads affected lymphocytes throughout the body.

Superior and inferior mediastinum

Primary lymphomas of this type are characterized by the action of predominantly sluggish (indolent) diseases. In this usually non-Hodgkin type of disease, lymphocyte dysplasia is observed. This is where the most common type of disease is found.

But there are, however, rare cases when the disease becomes aggressive. In such cases, she can settle in:

• trachea;
• bronchi;
• pleural cavity;
• esophagus

Causes

Medicine cannot answer this question. It only notes trends in the growth of diseases that are not Hodgkin's types around the world.

As researchers of this disease say, poor nutrition is to blame. Especially now, more and more products are being produced in which chemical additives predominate.

Vegetables are not excluded from this list. They are known to contain a lot of pesticides. The environmental situation contributes to the development of a disease such as mediastinal lymphoma. In addition to these reasons, we can name the determining factors that cause people to get sick.

And diseases begin due to:

1. radiation exposure;
2. contact with carcinogens;
3. pesticides, which are abundant in the agricultural industry;
4. aggressive viruses that are aimed at destroying the immune system;
5. autoimmune diseases;
6. age characteristics.

Children suffer from such a disease as mediastinal lymphoma very rarely. These diseases mainly affect males and females in middle and young age. In this disease there are periods, both asymptomatic and with pronounced clinical manifestations.

Tumor of the posterior mediastinum

The course of the asymptomatic period depends on:

• tumor location and parameters;
• malignancy or benignity of the tumor;
• its development;
• relationships with organs that belong to the mediastinum.

Typically, such a disease of the mediastinal organs can only be detected during a routine examination or x-ray.

Due to the fact that the symptoms of the mediastinum are not detected for a long time, the disease proceeds quietly and unnoticed at first, nothing bothers the person.

These diseases are primary and secondary in nature. When a person gets sick for the first time, the disease develops precisely in the mediastinum.

With a secondary disease, metastases can disturb the rest of any organ. Due to the fact that the disease takes on a common malignant process. With secondary lesions, lymph flows out from the diseased area of ​​the body.

All symptoms of the disease are associated with its localization. If the lungs and respiratory organs have taken over the outbreak, then symptoms are observed in the form of:

1. shortness of breath;
2. cough;
3. sputum discharge with blood;
4. pain during breathing (sternum area).

Cytostatics work well in the treatment of drugs together with Rutuximab. The drug works great thanks to monoclonal antibodies. In general, it affects the tumor with minimal side effects.

Chemotherapy helps achieve good results. As a result, malignant areas of the affected organs are destroyed.

They have an auxiliary principle, although these methods have also proven themselves on the positive side.

In some cases, surgical treatment is suggested. Tumor tumors are excised from patients. Typically, surgical interventions are rarely performed. Radiation may be performed for direct medical reasons.

In addition to these types of treatment, biological therapy is used. Using this method, the patient has a chance of recovery. In this case, the manifestation of pathological processes only decreases. With this type of treatment:

• immune therapy is carried out;
• use gene therapy;
• treatment is carried out with angiogenesis inhibitors, which prevent the growth of new tumor formations.

Forecasts

Modern polychemotherapy and radiotherapy help achieve high results. Survival is predicted to be five years. And these results are observed in 95% of patients. They undergo a favorable pathological process.

The intermediate course of the disease is overcome by 75%, the unfavorable course by 60%. Only aggressive types of mediastinum cause deaths. But even with such sad data, something can be done if the patient seeks help in a timely manner.

If the oncological process is detected early, then lymphoma of this type will be cured very quickly. When other systems and organs are involved, the chances of cure will drop sharply.

If the diagnosis is made in a timely manner, the patient can be saved. The outcome of these diseases is directly proportional to the development of malignant processes and forms of diseases. When making a forecast, attention is paid to a number of factors.

Some of them are:

1. age category of patients;
2. the degree of the affected immune system;
3. stage of the affected lymphatic system;
4. how the disease develops.

According to the prognosis, this species can be successfully treated in the first stage. 10 years after the illness, 90% survival rate. If the circulatory system or bone marrow is affected, the survival rate drops sharply and amounts to 20%-25%.

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