Acute leukemia last stage of care. Acute leukemia: symptoms, treatment and prognosis

Chronic leukemia is a tumor disease of the hemosynthesis system, the initial product is mature or maturing cells of the lymphoid or myeloid type. The manifestation of obvious symptoms is possible: intoxication, thrombohemorrhagic, lymphoproliferative.

The exact causes of leukemia are not known. The most common theory of hemoblastosis is associated with viral genetic origin. According to it, in connection with some viruses (retroviruses, Epstein-Barr virus), they penetrate into normal cells, provoking their uncontrolled division (cloning). Genetic predisposition also plays an important role. And if the first reason indicated is only an assumption, then in the second case it is reliably proven information. Leukocytosis is familial and hereditary. Chronic myeloid leukemia is associated with pathology on chromosome 22, a fragment of the long arm is transferred to chromosome 9.

The most common predisposing factors that cause leukemia are:

• radiation exposure in high doses;
• harmful chemical production;
• taking certain medications;
• bad habits (smoking, alcoholism).

The risk of developing a lymphocytic form of leukemia increases with constant exposure to pesticides and herbicides; the myeloid form can develop due to radiation exposure.

Types of leukemia

The division of leukemia into types occurs depending on the cellular substrate and origin.

Lymphocytic leukemia

It is more common in people over 40 years of age. Men are affected 2 times more often than women. During the examination, the following symptoms clearly predominate:

• anemia (often autoimmune);
• lymphadenopathy;
• granulocypenia;
• thrombocypenia;
• immunosuppression;
• predisposition to viral and infectious diseases.

The prognosis of the disease is good. The course is long, but with a successful outcome.

Myelocytic leukemia

Contains tumor cells such as cytic or procytic precursors of the myeloid series. Malignant transformation possibly occurs in a pluripotent stem cell.

This type of disease is the most interesting to study. It is not so rare, it occurs in about 15% of cases of all leukemia. The occurrence is diagnosed in people of any age, but predominates in the male population. The causes are the same as the acute form of the disease.

Monocytic leukemia

A blood test reveals a higher level of monocytes. In this case, the level of leukocytes is normal or not very pronounced. Promonocytes also enter the bloodstream, which leads to decreased immunity and secondary infection.

This disease is caused by several factors:

• decreased blood clotting;
• anemia;
• frequent causeless bleeding;
• oxygen starvation.

With timely and correct treatment, the prognosis is quite good. But otherwise, death is possible.

Stages of development of pathology and symptoms

There are three stages of chronic leukemia, each of which is accompanied by certain symptoms.

initial stage

The initial stage of this disease is not detected or it happens by chance during a blood test. Clinical data are weakly expressed or not manifested at all. The chronic form of the disease proceeds very slowly, and this stage may go undetected for several months.

Expanded stage

The advanced stage is already marked by a large number of symptoms, which are mainly noticed in a blood test. Patients experience rapid fatigue, increased sweating, a sudden increase in temperature, sudden weight loss, pain when moving in the left side due to an enlarged spleen, enlarged lymph nodes, liver, hemorrhagic syndrome (in rare cases), arrhythmia, pain in the heart area. , pneumonia.

Terminal stage

Symptoms, which were not so obvious in the advanced stage, increase and become stronger in the terminal stage.

• the temperature increase becomes more stable;
• the hemorrhagic symptom manifests itself in most cases;
• resistance to treatment;
• the process goes beyond the bone marrow;
• metastasis to different parts of the body;
• internal bleeding;
• radicular pain;
• secondary infectious complications are possible;
• hematological changes are similar to acute leukemia.

Features of the manifestation of chronic leukemia in children

Chronic leukemia begins its development very slowly. His symptoms are also hidden, which makes it impossible to diagnose the disease in a timely manner. Young patients may complain of severe fatigue, pain in joints and bones, general weakness and discomfort in the spleen area.

In some cases, there are no complaints from the patient at all. Therefore, detection occurs during a blood test during medical examination, before a trip to a sanatorium, camp, or after another illness. At the same time, in older children the development of leukemia occurs quickly and rapidly, in children it is slow and prolonged. The course of the disease also occurs at different rates depending on the age category. In older children and adolescents, the symptoms are less pronounced, while in younger children, fever and other striking manifestations are often observed.

Diagnostics

Initially, the doctor conducts a survey to draw up a clinical picture.

A hemogram is also analyzed, with the result of which the patient is referred to a hematologist. With myeloid leukemia, typical symptoms appear: anemia, the presence of single granulocytes and myeloblasts in the blood test. During a blast crisis, cells of the same name can reach 20% or more. Also, in the chronic form, a hematological study can detect increased level lymphocytes, leukocytes, lymphoblasts, Botkin-Gumprecht cells.

To determine the origin of the tumor substrate, examinations are carried out:

1. Wall puncture - performed in case of cytopenic syndrome. The procedure is done to assess the state of bone marrow hematopoiesis, exclude blood diseases, and to carry out differential diagnosis with hemaplastosis.
2. Trephine biopsy - bone marrow with part of the spongy and compact bone substance is removed for analysis. Indications for use are also cytopenic syndrome of unknown origin. However, a contraindication is a blood clotting disorder, so it is used only after a hematological study.
3. Lymph node biopsy – performed if metastasis that has spread to the lymph nodes is suspected.
4. Bone marrow punctate.
5. Ultrasound of lymph nodes, spleen.
6. Lymphostincigraphy.
7. Chest X-ray.
8. Multislice computed tomography of the abdominal cavity.

Biopsy is one of the methods for diagnosing chronic leukemia

It is also possible to use other examination methods for certain symptoms and accompanying diseases.

Method of treatment

At initial stage the disease does not require treatment. The patient is under dynamic observation. It is recommended to avoid excessive physical activity, stress, heat therapy, electrical procedures, etc. It is necessary Fresh air, walks, proper nutrition.

Drug treatment

In the advanced stage, chemotherapy is prescribed using drugs containing hydroxyurea, busulfan, mitobronitol, etc.

The following schemes can be used:

• SOAR - Cytosar, Cyclophosphamide, Prednisolone, Vincristine;
• CHOR - Prednisolone, Cyclophosphamide, Vincristine, Adriamycin;
• ROMP – Prednisolone, Vincristine, Methotrexate, Purinetol.

Cytosar is a drug used in the treatment of chronic leukemia

If a transitional stage to a terminal stage is identified, then high-dose chemotherapy is used. Leukapheresis procedures are performed in combination with chemotherapy.

Radiation therapy

If the spleen is significantly enlarged (splenomegaly), localized irradiation of the affected organ is performed. Although this therapy does not lead to complete recovery, it helps to inhibit the development of the disease and delays the blast crisis.

It is also possible to irradiate lymph nodes and skin.

Transplantation

Complete cure is most often achieved with a bone marrow transplant. This is done using your own material or donor material. In this case, a donor can be used from close relatives or from a person close in analysis. The material is collected from the ilium. After it is filtered and processed, hematopoietic cells are injected intravenously.

Stem cell transplantation is currently being performed. This technique has not been fully studied and its effectiveness has not been confirmed.

Unconventional methods

Non-traditional folk methods are used only in combination with proven methods and after consultation with the attending physician. Herbal medicine plays only a supporting role. Under no circumstances should you use it yourself.

Often used:

• sage;
• mint;
• calendula;
• Melissa;
• corn silk;
• marsh calamus and others.

Complications

The course of the disease can be complicated by:

• vascular thrombosis;
• hemorrhagic syndrome;
• endogenous uricemia and uricosuria.

Neuroleukemia is one of the most severe complications of chronic leukemia. More often occurs with the lymphoblastic form of leukemia in children. Less common in other forms. The occurrence of complications is caused by metastasis of cancer cells into the membranes of the spinal cord or brain.

The onset of purulent-necrotic processes is also possible - trophic ulcers, tonsillitis, pneumonia.

Due to displacement by affected cells, it leads to the following consequences:

• anemia – pale skin, decreased performance, shortness of breath, chest pain;
• thrombocypenia – reduced ability of blood clotting, formation of small hemorrhages on the skin, which gradually occupy larger and larger areas; when damaged, heavy bleeding occurs.

Forecast

A favorable or unfavorable prognosis can only be determined by the correlation of many factors: gender, age of the patient, concomitant diseases, complications, characteristics of cancer cells, etc.

When diagnosed, patients are divided into two main risk groups: standard and high. The first group mainly includes children under 2 years of age and over 10 years of age, with accompanying symptoms - enlargement of lymph nodes by 2 cm, enlargement of the liver and spleen by 4 cm.

The course of the disease is quite long, so even without treatment the prognosis is quite favorable. But in the absence of timely treatment, the course of the disease can develop into an unpredictable form. In this case, doctors do not give guarantees.

With modern treatment, complete remission is achieved in 90% of cases. Moreover, 75% have more than 5 years of positive dynamics without relapses.

If the duration of complete remission is observed in patients longer than 6-7 years, then the disease is considered completely cured.

Despite high achievements in medicine, leukemia is still one of the most dangerous diseases. Treatment of the disease is complicated by late diagnosis. The first signs of leukemia in women are easily disguised as other diseases. Obvious clinical symptoms appear in the late stages of the disease, which are difficult to treat. In this article we will discuss in more detail the symptoms of leukemia in women.

Clinic

Although this disease occurs in different types and the forms of symptoms in women are very similar. Between themselves they differ in the degree of severity of clinical manifestations, different periods of remission and individual characteristics body. Thus, if the disease is in acute form, then it is very easy to identify. In turn, the chronic form has a latent course and a person may not even suspect that he is sick for years.

Women suffering from leukemia may present with a wide variety of complaints. Some of them may not directly indicate pathological processes in the hematopoietic system. For clarity, we have grouped all the characteristic signs of leukemia in women. Each of them indicates similar pathological processes in individual organs and systems:

1. General state. Sick women very often complain of a sharp, causeless loss of body weight, lack of appetite and an increase in body temperature for no obvious reason. Representatives of the fair sex with leukemia are apathetic, absent-minded, and unable to concentrate their attention for a long time. In severe cases, there is confusion and problems with coordination of movement.
2. Lymphatic system. There is an enlargement of the lymph nodes. This phenomenon is a violation of lymphatic drainage, which occurs due to a huge number of immature forms of leukocytes.
3. Respiratory system. The appearance of shortness of breath without physical activity in the absence of diseases of the respiratory and cardiovascular systems.
4. Sensory system. There is a decrease in visual acuity. Many patients complain of blurred vision.
5. Genitourinary system. Characterized by impaired urinary function, the appearance of swelling in the groin area, which is manifested by painful sensations.
6. Digestive system. Women complain of impaired taste, nausea, aversion to food and the appearance of gag reflexes.
7. Musculoskeletal system. Very often, people suffering from this disease experience pain in the joints and hands. Cases of convulsive seizures are common.
8. Leather. It takes on an unnatural reddish tint and flaky spots may appear. Very often, women complain about the appearance of bruises, nose and tooth bleeding for no reason. Sweating may increase at night.
9. Liver and spleen. Leukemia is characterized by hepatosplenomegaly.

The first symptoms of leukemia

All the first signs of leukemia appear after deterioration in normal functioning immune and hematopoietic system. First of all, there is a change in the qualitative and quantitative composition of biological fluids. This pathological condition then affects the work and functioning of internal organs and tissues.

Change quality composition blood leads to the development of anemia.

It is very difficult to identify the disease based on the first symptoms. This is due to the fact that most of the symptoms of the disease are very similar to other diseases and do not have specificity. The appearance of bright symptoms of blood cancer occurs when the disease begins to progress rapidly.

Stages of leukemia

There are 4 stages in the development of the disease. Each of them has its own clinical manifestations. The first symptoms of leukemia are very difficult to differentiate. Thus, the disease remains invisible. This occurs before the period of obvious clinical manifestations. Patients with chronic leukemia may have no symptoms at all. In this case, a person learns about his disease in the later stages of hemoblastosis.

Stages of blood cancer:

• Stage 1. An uncontrolled increase in the population of immature forms of blood cells leads to disruption of normal functioning immune system. Weakened immunity is fraught with the development of frequent infectious diseases, drowsiness and fatigue. The first stage of the disease is characterized by frequent relapses of respiratory system diseases and the appearance of shortness of breath.
• Stage 2. Modified blood cells accumulate in the organs and tissues of the body. Tumor-like formations are formed from immature forms of leukocytes. At stage 2 of blood cancer, bruises, hemorrhages, hemorrhagic rashes, enlarged lymph nodes, hepatosplenomegaly and inflammatory processes in the oral cavity occur.
• Stage 3. The rapid process involves the entire body and leads to serious disruption in the body. At this stage, symptoms can easily make a preliminary diagnosis. Representatives of the fair sex complain of nausea, lack of appetite, sudden loss of body weight, bloating in the abdomen, headaches, nosebleeds, low-grade body temperature, decreased visual acuity, vomiting, bone and joint pain. At stage 3, women very often complain to the gynecologist about painful and heavy menstruation. Uterine bleeding may also occur.
• Stage 4. The last stage of the disease is characterized by metastasis and proliferation of the malignant neoplasm. The patients stay in depressed state they are worried about constant fever, pain in the heart, frequent urination, external and internal bleeding, as well as cutting pain in the abdomen.

Blood cancer classification

Leukemia is an oncological disease that leads to mutation of bone marrow cells. In hemoblastosis, the maturation of blood cells is disrupted. This process results in the replacement of healthy cells with cancerous ones. Hemoblastosis is usually divided into types. The classification is based on the type of damaged cells in the hematopoietic lineage. Symptoms will differ depending on the type of cells affected.

The lymphoblastic type in its acute form occurs between the ages of 3 and 7 years. It is characterized by symptoms of anemia, intoxication, respiratory and renal failure. There is an enlargement of the lymph nodes.

Acute myeloblastic leukemia occurs in people over 55 years of age. Anemia is the first sign of leukemia in women. Patients are worried about constant fever, osteoarticular pain and uterine bleeding. On examination, conglomeration of lymph nodes is observed.

Lymphocytic leukemia can appear at any age. It is characterized by a sharp enlargement of the lymph nodes, which may arise as a result of a history of infectious disease. On examination, hepatosplenomegaly and periodontal disease are detected.

Patients are concerned about pain in the right hypochondrium.

Chronic myeloma leukemia most often affects people aged 30 to 50 years. The progressive stage of the disease can last from 2 to 10 years. Patients constantly complain of shortness of breath, sharp pain in the right hypochondrium, headaches, bone and joint pain. There are frequent cases of disturbances in orientation and coordination in space.

conclusions

If you have discovered symptoms of leukemia in yourself or your loved ones, then do not panic. At possible signs illness, you should immediately consult a doctor to confirm or refute the diagnosis. Only a doctor, based on the first manifestations and additional research methods, can correctly determine the real reason illness and prescribe effective treatment.

In contact with

At the Israeli Center for Blood Diseases at the private clinic "Herzliya Medical Center" treatment of leukemia at any stage.

When it comes to cancerous tumors formed in any organ, the stages of cancer are classified into four clinical degrees diseases. Under this system, cancer is staged based on the size of the tumor and its spread to other organs.

Due to the fact that the development of leukemia occurs in the bone marrow, after which the disease spreads to other organs until it is detected, there is no need to use the traditional classification of the stage of the disease. Instead, doctors use a cytology grouping system to determine the type and subtype of leukemia. Classification of tumor cells allows one to predict the prognosis of specific forms of leukemia and the likely response of the disease to treatment.

The most popular classification method is the Franco-American-British (FAB) system. According to this classification, acute leukemia is divided into eight subtypes of acute myeloid leukemia (AML) and three subtypes of acute lymphocytic leukemia (ALL). Initially, the FAB system was based on the microscopic appearance of leukemia cells, but in last years Research has shown that cellular characteristics, such as the genetic composition and abundance of individual cell types, allow leukemia to be classified and produced.

Classified according to one of the cytological staging systems known as Rai Classification and Binet Staging.

Determination of the stage of leukemia according to the Rai Classification

According to this classification, chronic lymphocytic leukemia can have low, moderate and high degree risk, each of which respectively includes stages 0, I, II, III and IV:

• Stage 0: Low-risk patients with lymphocytosis and high lymphocyte counts (more than 15,000 lymphocytes per cubic millimeter);
• Stage I: Patients are at intermediate risk, characterized by lymphocytosis and enlarged lymph nodes (lymphadenopathy);
• Stage II. Patients are at intermediate risk but also have lymphocytosis and an enlarged liver (hepatomegaly) or an enlarged spleen (splenomegaly) with or without lymph node enlargement;
• Stage III. Patients are at high risk and have lymphocytosis with anemia (low red blood cell count) with or without lymphadenopathy, hepatomegaly, or splenomegaly.
• Stage IV (terminal stage of acute leukemia). Patients have high risk, lymphocytosis, thrombocytopenia (low platelet count in the blood).

Determination of the stage of leukemia using the Binet method

Binet's formulation classifies chronic lymphocytic leukemia depending on the number of lymphoid tissues involved in the pathogenic process (spleen, lymph nodes of the neck, groin, armpits), as well as the presence of a low number of red blood cells (anemia) or platelets in the blood (thrombocytopenia).

Stages of leukemia according to Binet's classification:

• Stage A: Patients have less than three areas of enlarged lymphoid tissue and do not have anemia or thrombocytopenia. Enlarged lymph nodes on one or both sides of the neck, groin and armpits, as well as the spleen, are considered separate areas.
• Stage B. There are three areas of increased lymphoid tissue; There is no anemia or thrombocytopenia.
• Stage C. There are more than three areas of increased lymphoid tissue, anemia and/or thrombocytopenia. Typically, such symptoms indicate the terminal stage of acute leukemia

Clinical features of leukemia stage classifications

One of the major advantages of the Binet system is its ability to identify a form of CLL that may have a better prognosis than the Rai classification system.

There are several other types and subtypes of chronic leukemia, the most famous of which are hairy cell leukemia, prolymphocytic leukemia, T-cell chronic lymphocytic leukemia, and chronic myelomonocytic leukemia.

Hairy cell leukemia, like CLL, is a slow-growing lymphocytic cancer that is identified under a microscope (depending on the surface of the cells, specialists can make a prognosis). Hairy cell leukemia typically affects middle-aged men. However, most patients report nonspecific symptoms, partly caused by anemia. However, hairy cell cancer differs from CLL in symptoms, treatment, and diagnosis.

Prolymphocytic leukemia (PLL) and T-cell chronic lymphocytic leukemia (T-CLL) are uncommon forms of CLL and are quite rare (accounting for 1% of all CLL cases). PLL is more common in men. More than half of cancer cells are prolymphocytes, which are larger in size compared to CLL cells and have less condensed genetic material.

T-CLL is a more aggressive form of cancer by nature compared to other forms of CLL and is less responsive to treatment.

There is some disagreement among scientists about the categorization of chronic myelomonocytic leukemia (CMML). Some experts consider it a "pre-leukemia" or "myelodysplastic syndrome" rather than a malignant disease. The disease most often occurs in older people, is diagnosed incidentally and may be asymptomatic. Patients report symptoms of fatigue, bruising, and abdominal discomfort caused by an enlarged spleen.

The disease may remain inactive for a long time and progress very slowly over months or even years, then eventually develop into acute form leukemia, usually with a fatal outcome (that is, the patient is diagnosed with the terminal stage of acute leukemia).

Leave your contact details and the center consultant will contact you to organize a consultation with a hematologist

Leukemia is a malignant neoplasm of the bone marrow in which, depending on the stage of leukemia, atypical blast cells are formed. In structure they resemble the precursor cells of leukocytes, but do not perform their function.

The formation of lymphoblasts occurs many times faster than normal leukocytes. At a certain stage of leukemia, they replace most of the normal white blood cells, leading to severe consequences, such as anemia, bleeding and infection. They migrate with the blood, creating lymphoblastic foci throughout the body.

Acute leukemia: stages

Onset of the disease - the initial stage of acute leukemia has many symptoms. In the general analysis of red blood, no special changes are noted. In rare cases, a decrease in the number of red blood cells and hemoglobin can be detected. In white blood, either a decrease or an increase in the level of leukocytes is detected. The number of “blast” cells exceeds the norm, slightly. Sometimes you can see thrombocytopenia - a decrease in platelet counts. A bone marrow biopsy is more essential for diagnosing leukemia. At the same time, a significant increase in the number of progenitor cells is found in the bone marrow. The early stage of acute leukemia is almost always diagnosed at the end, when it progresses to the next stage.

Stage of full clinical development

Its first signs are most often detected in a blood test. It is characterized by a significant increase in immature forms of leukocytes in the blood and the appearance of “blast” cells. There are several clinical options for this stage:

• the patient feels well, does not complain about anything, but characteristic changes are detected in the blood test;
• The patient has some complaints, but the blood test is normal.

The stage of full clinical development is manifested by three syndromes:

1. Infectious-toxic. Inflammation occurs in different organs.
2. Hemorrhagic. There is a tendency to bleed. In this case, significant blood loss is possible.
3. Anemic. The amount of hemoglobin and red blood cells decreases. The skin and mucous membranes turn pale, the patient complains of increased fatigue, a feeling of lack of air, and dizziness. The work of the heart is defective.

Remission stage

In this case, remission can be complete or partial. The stage of complete clinical remission is characterized by the absence of a clinical picture and complaints, and no more than 5% of “blast” cells are found in the bone marrow tissue. Blood test without pathological changes. If remission is partial, the patient feels well, and more than 5% of immature pathological cells are observed in the bone marrow.

Relapse stage

An increase in the content of “blast” cells in the blood and bone marrow, against the background of the patient’s general poor health, indicates a return of leukemia. Each subsequent relapse is more dangerous than the previous one and more often causes complications. The relapse stage ends in terminal or goes into remission.

Terminal stage

The last stage is characterized by polyclonal progression of the tumor and pronounced malignancy of the process. Almost all normal cells are replaced by pathological ones, which leads to complications and serious condition of the patient. Ulcerative-necrotic processes are observed in many organs.

The stages of leukemia, in the acute period, are very similar, they have more common symptoms than differences. But to prescribe adequate treatment great importance has a definition of exactly what stage leukemia is in.

Stages of chronic leukemia: initial

The period preceding the deployment of the clinic lasts from six to twelve months. In the early stage of chronic leukemia, the patient often does not feel like a sick person, but minimal deviations are detected in a blood test. In rare cases, patients feel:

Every third person has an expansion of the boundaries of the liver and spleen. In the blood, neutrophilic leukocytes are several times higher than the normal value, and the level of platelets increases. An important diagnostic criterion is determining the amount of alkaline phosphatase in leukocytes. In chronic leukemia, its amount is several times less than normal.

The number of red blood cells and hemoglobin are within normal limits. ESR is often normal; its acceleration is extremely rare. The platelet count is slightly higher than normal. When examining bone marrow puncture, a slight increase in the number of immature leukocytes can be detected.

Stages of chronic leukemia: advanced

The period in which the disease is diagnosed not only by blood tests. It has a clinical picture characteristic of acute leukemia. In the advanced stage, there is an almost complete inhibition of the formation of normal blood cells. The patient's condition worsens. It can last from three to five years, sometimes even longer.

A distinctive feature is the detection of an enlarged spleen. Sometimes it increases to such gigantic in size that the doctor can feel it in the pelvis. The abdomen is significantly increased in size. The patient notes a feeling of heaviness in the abdomen due to compression of neighboring organs by the spleen. The liver also enlarges. But its size is still several times smaller than the spleen.

One of the first signs of this stage may be general weakness, sweating, and loss of body weight. Sometimes the patient notices a constant increase in temperature. The patient is emotionally unstable, it is difficult for him to work, and he sleeps poorly. In some cases, there may be allergic reactions on the skin. Bones and joints begin to ache.

The level of leukocytes is sharply increased, blast cells are found in large numbers in the peripheral blood. Many immature forms are detected. The level of lymphocytes is significantly reduced. The number of eosinophils also increases. Platelets are within normal limits or elevated, which is very rare. ESR accelerates. Many “blast” cells are found in the bone marrow tissue.

Stages of chronic leukemia: terminal stage

The steady progression of the disease ends in a terminal period. In the last stage, tumor cells fully demonstrate their malignancy. The patient's condition is severe or very serious. Malignant cells leave the bone marrow and travel through the bloodstream throughout the system and settle in vital organs.

The terminal stage comes very unexpectedly. If treatment is ineffective, the patient lives for a maximum of six more months. It begins with a sharp increase in the level of white blood cells. The treatment that helped before no longer works. Complete remission can be achieved only in every fifth patient. In most cases, partial remissions are observed, lasting a maximum of a month.

The patient continues to lose weight and become exhausted. Due to anemia, the skin is pale. Possible heart damage in the form of arrhythmias. On the skin, so-called leukemids are formed from “blast” cells. They are very painful, the skin over them is slightly red. Lymph nodes increase in size. The bones and joints hurt so much that the patient cannot even fall asleep without painkillers. As a result of the almost complete absence of immunity, severe inflammatory processes of parenchymal organs are possible.

"Blast" crisis is characteristic feature critical stage. With it, the number of malignant cells in the blood exceeds 10%, and in the bone marrow - 20%, and this figure is constantly growing. The disease can progress slowly over a long period of time and remain in a slow-moving state for decades. But then it goes into a rapid terminal stage, which ends in death.

Experience shows that in the initial stage of leukemia there are no clinical manifestations: patients feel completely healthy until the widespread spread of tumor cells throughout the hematopoietic system, until the development of organ dysfunction due to tumor growths.

Diagnosis of acute leukemia can only be established morphologically - by the detection of undoubtedly blast tumor cells in the blood and bone marrow. Any specific external signs characteristic of initial period acute leukemia cannot be detected.

Hematological picture of acute leukemia can be twofold. When blast cells enter the blood, the leukogram reveals a pattern characteristic of acute leukemia: the presence of both young blast cells and mature granulocytes, monocytes, and lymphocytes. Even if promyelocytes and myelocytes are found in a blood smear, their percentage in acute leukemia is small and the picture of a gap in the leukogram between young and mature cells remains. If blast cells have not yet acquired the ability to exit the bone marrow into the blood, but have already led to some disturbances in the body, then their content in the bone marrow is quite high. In this case, leukopenia, anemia, thrombocytopenia or pancytopenia are observed in the blood.

In all cases of repeatedly detected unexplained cytopenia, bone marrow puncture examination should be considered mandatory. In acute leukemia, almost always, if there is cytopenia in the blood, the bone marrow contains tens of percent blast cells. An exception to this rule may be rare cases of protracted onset of acute leukemia, when blast cells have a pronounced cytopenic effect, but have not yet had time to produce significant proliferation; in the same way, when autoimmune cytopenia appears from the very beginning of acute leukemia (autoimmune cytolytic conditions can complicate the course of any leukemia), the percentage of blast cells in the bone marrow may be small.

Certain difficulties in making a diagnosis of acute leukemia are presented by the so-called low percentage acute leukemia, characterized by a low content of blast cells in the blood (less than 10-20%) and sometimes even less blastosis in the bone marrow. Diagnosis of this relatively rare acute leukemia, which occurs mainly in old age, is not so difficult, since blast cells in the peripheral blood in an amount of several percent are not found under any reactive conditions.

A characteristic diagnostic sign of acute leukemia is the classic structure of the nucleus of blast cells - delicately chromatic, finely reticulated, with uniform color and caliber of chromatin threads.

At the same time, leukemic blasts are very heterogeneous even in the same patient in one smear.

The described typical forms of blasts are observed relatively rarely; the bulk of tumor cells consists of elements either with a smeared chromatin structure, but with cytoplasm similar to the typical blasts of this preparation, or with a rough, irregular chromatin network, but with nucleoli, etc. All such cells with When counting, myelograms or hemograms can be classified as blasts only when true blasts make up tens of percent. In all other cases, atypical blasts cannot be classified as such - they should be described in detail without giving them a name.

Of particular importance is correct assessment of the nature of cells in the diagnosis of remission of acute leukemia, which is characterized by the presence of 5% blasts or less. In this case, it is very important to focus on the content of myelokaryocytes in the smear: with a small number of cells (for example, with agranulocytosis), young lymphoid cells with narrow cytoplasm, sometimes with nucleolus (especially in children), but with a homogeneous chromatin structure, may predominate. These are progenitor cells. They should be classified as lymphoid cells. The clumpy structure of normal lymphocytes is not typical for them. The detection in the bone marrow of more than 20-30% of round-nuclear cells resembling blasts is usually regarded as the appearance of atypical cellular elements requiring an accurate morphological description.

The use of cytostatic drugs and prednisolone before establishing a diagnosis is unacceptable, as it can lead to erroneous diagnosis of acute leukemia in cases of infectious mononucleosis or immune hemolytic crisis.

Sometimes acute myeloblastic leukemia begins with an increase in the content of all young cells in the blood: blasts, promyelocytes, myelocytes, metamyelocytes, etc.

Differential diagnosis of blast crisis of chronic myeloid leukemia and acute myeloblastic leukemia doesn't have much practical significance, since the therapy is the same in both cases.

Significant Diagnosis may be difficult due to acute immune hemolysis, which is accompanied by a sharp increase in the content of reticular cells in the bone marrow (up to 10-20%). These cells are sometimes mistaken for blasts, although they always have a rough chromatin structure and large blue nucleoli; in addition, a sharp increase in the number of bone marrow erythrokaryocytes is detected, and high reticulocytosis in the blood.

It is also possible that, along with a low percentage of blast cells in the bone marrow punctate, there is a break in the maturation of granulocytic cells at the level of myelocytes or promyelocytes, which is more often observed with immune neutropenia or agranulocytosis. The difficulty in diagnosis is especially great if there are no blasts in blood tests, no thrombocytopenia, and the blast cells in the bone marrow punctate do not differ in atypia and, in terms of the ratio of nucleus and cytoplasm, the absence of granularity in the cytoplasm, resemble normal precursor cells in the form of a blast. In such doubtful cases, trephine biopsy is indicated, which makes it possible to detect proliferation of blast cells in acute leukemia.

These difficulties in establishing the diagnosis of acute leukemia are relatively rare and should not serve as a reason for unlimited puncture of the bone marrow, for example, in patients with transient leukopenia after influenza, with stable leukopenia against the background of thyrotoxicosis or cirrhosis, etc.

There may be situations when, due to cytopenic syndrome, it is not possible to make a diagnosis, although acute leukemia is suspected. In these cases, all studies have to be repeated after several weeks or months. Some of the delayed diagnoses should be attributed to shortened blood tests, when the number of platelets and reticulocytes is not calculated in case of anemia of unknown nature.

At the onset of acute leukemia normochromic or somewhat hyperchromic anemia may be observed - the color index reaches 1.2-1.3. The tendency to hyperchromia is especially pronounced in acute erythromyelosis. Among the erythrocytes there is a significant number of macrocytes. The platelet count is either low or normal in most cases. Very rarely there are cases of acute leukemia (apparently non-lymphoblastic) with hyperthrombocytosis reaching several million in 1 μl. At the same time, platelets of unusual shape, large sizes with blue cytoplasm are also detected.

Regarding the problem of the initial manifestations of acute leukemia, it is necessary to emphasize that in all observed cases in which it was possible to detect an aneuploid clone, or a clone with some defect in the karyotype, after one or another period of time it was this clone that always became the basis of blast leukemia.

If an abnormal cell clone is detected in a patient with cytopenia, a diagnosis of the initial manifestations of acute leukemia should be made. In the case of a normal karyotype, a syndromic diagnosis is made:

• aplastic syndrome (aplastic anemia with focal hyperplasia of undifferentiated elements in the bone marrow);
• thrombocytopenic syndrome;
• granulocytopenia of unknown origin, etc.

One of the signs of future acute leukemia may be unmotivated monocytosis, which, unlike chronic monocytic leukemia, is not accompanied by polymorphic-cellular pronounced hyperplasia of cells in the bone marrow trephine. The nature of such monocytosis, which often appears several years before the development of leukemia, is not entirely clear. Since in such cases of prolonged monocytosis myeloblastic or myelomonoblastic acute leukemia, or acute erythromyelosis against the background of previous neutropenia, then develops, one can think that monocytes in these cases are not leukemic, but arise as a result of regulatory disorders of monocytopoiesis due to leukemic suppression of neutrophilopoiesis (similarly reactive monocytosis develops with hereditary neutropenia).

The number of leukocytes at the beginning of the process is most often reduced, but at the same time there are cases when, at the first clinical manifestations, high leukocytosis is noted with a predominance of blast cells in the hemogram.

Often in acute leukemia, single erythrokaryocytes are found in the blood, which have significant differential diagnostic significance: they are absent in reactive conditions (excluding hemolysis, leukemoid reactions to cancer), infectious mononucleosis.

In some very rare cases, erythrocytosis (more than 5T in 1 liter) is observed, preceding the full picture of acute leukemia. If the release of erythrokaryocytes, as well as the appearance of myelocytes and promyelocytes in the blood, can be associated with a violation of the structure of the bone marrow, the proliferation of blasts (a similar blood picture occurs with cancer metastases to the bone marrow), then macrocytosis, hyperchromia of erythrocytes, as well as the often observed phenomenon of megaloblastoidity erythrokaryocytes are probably associated with defects in the red progenitor cells, which, already being leukemic, retain some ability to differentiate.

ESR in acute leukemia may be slightly increased, sometimes significantly increased or normal.

The described blood picture is associated with the primary process caused by leukemia, and changes significantly under the influence of cytostatic therapy; Moreover, it is not the same various forms acute leukemia.

Stages of acute leukemia

Classification of the stages of acute leukemia has purely practical purposes: determining therapeutic tactics and assessing prognosis.

Currently, due to the success of cytostatic therapy for leukemia, clear boundaries of the stages of the process determine all treatment tactics. In some cases, we are talking about the use of powerful complexes of cytostatic agents aimed at eradicating leukemia, in others - about the prevention of relapses using a long-term but weak cytostatic effect, and in others, about the elimination of local relapse. But very often the fight to eradicate tumor growth becomes impossible and the doctor is forced to limit himself to only maintaining the achieved partial effect.

These fundamental differences in treatment tactics formed the basis classification of stages of acute leukemia that can be imagined in the following way: initial stage, first attack, or advanced stage of the disease, complete remission, recovery, partial remission, relapse indicating its number and location, terminal stage.

Initial stage of acute leukemia

The information accumulated to date about the initial stage of acute leukemia is so scarce that it is not yet possible to give a specific definition of it. Most often we are talking about a retrospective assessment, when, for example, a limited tumor of blast cells (in a lymph node, skin, meninges, etc.) with a normal composition of the bone marrow subsequently becomes contaminated with power cells and their release into the blood. However, if the lymphoblastic nature of isolated tumors of the lymph nodes in children is detected, it seems most appropriate to treat them according to the usual regimen for lymphoblastic leukemia.

Advanced stage of acute leukemia

The advanced stage of the disease is characterized by pronounced inhibition of normal hematopoiesis, high bone marrow blastosis (except for low-percentage leukemia). This stage is heterogeneous from a therapeutic and prognostic point of view:

• the first attack of leukemia is fundamentally different from the relapse that developed during cytostatic therapy;
• each subsequent relapse is prognostically more dangerous than the previous one, and usually requires a new combination of cytostatic agents (although there may be a return of sensitivity of leukemic cells to previously used cytostatics);
• in its turn, remission can be complete or partial.

Complete remission in acute leukemia

Complete remission includes conditions, in which no more than 5% of blast cells are found in the bone marrow aspirate, and the total number of blast and lymphoid cells does not exceed 40%, while there are no blast cells in the peripheral blood, the blood composition is close to normal, clinical signs of leukemic proliferation in the liver, spleen and other organs is not noted (for lymphoblastic acute leukemia, a mandatory criterion for complete remission is the normal composition of the cerebrospinal fluid).

Recovery from acute leukemia

Recovery from acute leukemia a state of complete remission for five years or more is considered.

Partial remission in acute leukemia

Partial remission is a rather heterogeneous group of conditions that are characterized by a clear hematological improvement (a significant decrease in the percentage of blast cells in the bone marrow with an increase in the percentage of normal cells, combined with an improvement in blood composition), or the disappearance of blast cells from the blood while maintaining bone marrow blastosis, or a decrease in the number of blast cells cells in the cerebrospinal fluid during elimination clinical symptoms neuroleukemia, or some suppression of other foci of leukemic proliferation outside the bone marrow, etc.

Relapse of acute leukemia

Relapse of acute leukemia may be bone marrow related(appearance of more than 5% blast cells in punctate) or local- extramarrow with any localization of leukemic infiltration.

Leukemic and aleukemic phases of acute leukemia

From a purely hematological point of view leukemic should be isolated(with the release of blasts into the blood) and aleukemic(without their appearance in the blood) phases of acute leukemia.

Regardless of the reason that caused the remission, the hematological and clinical picture of the disease is characterized by natural dynamics. If a patient has a leukemic phase of the disease, in the case of effective therapy, blast blood cells often lose the characteristic structure of nuclear chromatin and turn into lymphocyte-like ones. Sometimes this transformation takes 1-2 days, most often it takes several days.

If leukemia is accompanied by intoxication and hemorrhagic syndrome, then, despite the lack of growth of mature normal blood cells, the patient’s well-being improves with the onset of remission and bleeding decreases.

Subsequently, the number of leukocytes decreases (due to the predominant disappearance of pathological cells), leukopenia of varying degrees occurs, and then an increase in the number of mature normal cells is noted. In the leukemic phase of the disease, the stage of pancytopenia before the restoration of blood is almost mandatory.

The actual completeness of remission cannot be assessed. A biopsy of internal organs, the results of a pathomorphological study (in case of death from infectious complications), undertaken for splenectomy or for other reasons, show that the proliferation of undifferentiated blast cells in the spleen, kidneys, and lymph nodes persists in patients in a state of remission. Chromosomal analysis of the bone marrow also indicates the possibility of maintaining 0.5-1% of aneuploid cells (the same as before remission) during long-term (2 years) remission, although the myelogram remains consistently normal. Microscopy of punctate allows in some cases to identify single, undoubtedly pathological cells among blasts, which constitute less than 1% and do not interfere with assessing the situation as complete remission.

Sometimes the concept " clinical remission", which is characterized by improvement general condition patient, disappearance of septic complications, hemorrhages with minor changes in the hematological picture of the disease. In these cases, it is customary to talk about clinical improvement without remission.

Terminal exacerbation of leukemia often preceded by partial remission. In cases where pathological cells become less sensitive to all cytostatics used than normal parent cells of the bone marrow, i.e. when, under the influence of cytostatics, granulocytopenia or thrombocytopenia increases faster than the content of blast cells decreases, the doctor is forced to stop trying to achieve complete hematological remission and move on to treatment methods aimed at achieving a partial positive effect.

Terminal stage of acute leukemia

The terminal stage of acute leukemia at first glance does not have certain features, but observation of patients shows that in the development of leukemia there inevitably comes a moment when all cytostatic drugs not only turn out to be ineffective, but against their background the process progresses: granulocytopenia and thrombocytopenia increase, foci appear necrosis on the mucous membranes, spontaneous hemorrhages.

Manifestations of the terminal stage also include the appearance of foci of sarcomatous growth in the skin, myocardium, and kidneys.

However, the decisive role in the development of the terminal stage belongs primarily to the complete inhibition of normal hematopoietic germs, and not to lesions of internal organs, which may occur earlier and do not always mean the ineffectiveness of therapy (for example, in acute lymphoblastic leukemia, specific infiltration of the meninges or testicles is usually eliminated with prescribing cytostatics, gamma therapy, or, although it persists, does not lead to generalization of the process and death of the patient for a long time).

The concept of the terminal stage is conditional; it reflects only the current level of therapeutic capabilities and the incurable stage of tumor progression of leukemia.

Thus, The diagnosis of acute leukemia, indicating the stage of the disease, is formulated as follows: acute myeloblastic (lymphoblastic, promyelocytic, etc.) leukemia (complete remission; first relapse - bone marrow or local with testicular infiltration; partial remission - neuroleukemia with normalization of the bone marrow; terminal stage), aleukemic phase.

Any form of acute leukemia can occur with primary deep cytopenia. Isolating primary cytopenia into a separate stage is incorrect, since in all cases we are talking about an extensive process, but occurring with a pronounced inhibition of normal hematopoiesis. However, from a therapeutic point of view, this phenomenon deserves attention, since in some cases primary cytopenia is so pronounced that cytostatic therapy, by all formal criteria, seems contraindicated, although only it can lead to remission.