Acute lymphoblastic t cell leukemia. Acute lymphoblastic leukemia survival rate

• Which doctors should you contact if you have Acute lymphoblastic leukemia?

What is Acute lymphoblastic leukemia

Acute lymphoblastic leukemia

Acute lymphoblastic leukemia most often affects children. Its peak occurs at 2-4 years. Among adults, this form of acute leukemia occurs in 10-15% of patients.

Pathogenesis (what happens?) during Acute lymphoblastic leukemia

By the time acute lymphoblastic leukemia is diagnosed, the tumor is already quite large, and its mass usually reaches 1,1012 cells per 1 m2 of body surface and is about 3- 4% his body weight. This number of leukemia cells can be formed in 1-3 months of illness, while the number of leukemia cells in the bone marrow doubles in 4-10 days. By the time improvement occurs from prednisolone and vincristine, the content of leukemic cells decreases to 1 H 108 - 1 H 109 per 1 m2.

Symptoms of Acute lymphoblastic leukemia

The peculiarity of the clinical picture of this leukemia in children is frequent increase lymph nodes and spleen. Depending on the location of the predominant enlargement of the lymph nodes, the signs of the disease also change. When they are localized in the area chest Patients develop a dry cough and shortness of breath; enlarged mesenteric nodes can cause abdominal pain. Pain in the legs is typical. However, the onset of acute lymphoblastic leukemia in children is rarely accompanied by profound suppression of hematopoiesis; usually only normochromic anemia and moderate leukopenia are observed.

Diagnosis of Acute lymphoblastic leukemia

The clinical onset of the disease may coincide with the aleukemic and leukemic phases. Often there are nonspecific changes in the blood associated with a violation of the structure bone marrow: single erythrokaryocytes, myelocytes, promyelocytes - signs of myelemia. Bone marrow puncture, which reveals blast cells in a large percentage, resolves all diagnostic difficulties.

The morphology of blast cells has some features: the nucleus with a delicate chromatin network, like all blasts, is usually round, has 1-2 large nucleoli in many nuclei, the cytoplasm does not contain granularity. As with all acute leukemias, the shape of the nucleus changes during the course of the disease: it becomes irregular, its size increases; the rim of the cytoplasm also increases.

Specific histochemical features of this leukemia: blast cells do not detect peroxidase, phospholipids, esterases, and glycogen, detected by the PAS reaction, is distributed in the cytoplasm in clumps in the form of a necklace around the nucleus.

The study of T- and B-markers on blast cells of acute lymphoblastic leukemia showed that it represents a heterogeneous group. Available for at least There are 3 forms of this leukemia, identified by antigenic markers: acute lymphoblastic leukemia with blast cells that have B-lymphocyte markers, T-lymphocyte markers and no T- or B-lymphocyte markers.

The B-form of acute lymphoblastic leukemia includes leukemic stages of lymphosarcoma, Burkitt's lymphosarcoma, and very rare blast crises of chronic lymphocytic leukemia. Leukemia cells in this form are characterized by high density IgM on their surface.

Clinically, the features of the T-form of acute lymphoblastic leukemia have been more clearly studied. The form described above is most often detected in children of the older age group, while average age patients is 10 years old. According to most data, the average life expectancy of patients with this form of acute leukemia is less than 24 months, exacerbation in half of the cases begins with extramedullary growth - often with damage to the nervous system.

Blast cells in their own way antigenic structure resemble thymocytes and prethymocytes rather than peripheral T cells. Also, these cells retain some functional properties of suppressors. A cytochemical feature of T-blasts is the high activity of acid phosphatase and its localization in the cytoplasm.

The general characteristics of acute lymphoblastic leukemia refer predominantly to its neither T- nor B-form, comprising about 70% of cases.

According to antigenic and enzymatic characteristics, cells representing neither the T- nor the B-form are devoid of determinants of peripheral T- and B-lymphocytes. However, such cells have the features of thymocyte precursors: they react with antiserum to thymic antigens, with some antisera of chronic lymphocytic leukemia, and contain a lot of deoxynucleotidyl transferase. The next feature of these cells is that they have a lot of Ia antigen, characteristic of B cells.

In acute lymphoblastic leukemia, in addition to the 3 main forms identified by antigenic markers of lymphocytes (T-acute lymphoblastic leukemia, B-acute lymphoblastic leukemia and neither T- nor B-acute lymphoblastic leukemia), several more have been identified.

The new forms largely separated from neither the T nor the B forms. Thus, a pre-B form of acute lymphoblastic leukemia has been isolated: the blasts representing it belong to the early cells - the precursors of B lymphocytes, since they contain cytoplasmic immunoglobulin - the IgM heavy chain - and do not have immunoglobulins on the surface. The above-described form of acute, essentially B-cell leukemia has a significantly more favorable course than the B-form.

Leukemia, represented by lymphoblasts with the same antigenic markers, but containing the Ph chromosome, separated from neither the T nor the B form. This form of acute lymphoblastic leukemia occurs in older children - after 10 years; it progresses unfavorably, giving a short improvement.

In a small percentage of cases, acute lymphoblastic leukemia occurs, the blasts of which, according to their immunological characteristics, belong to pre-T-lymphocytes, the precursor cells of T-lymphocytes. In contrast to the pre-B form of acute lymphoblastic leukemia, pre-T, like other T-cell acute leukemias, is an unfavorable form.

T-cell forms of acute lymphoblastic leukemia may be accompanied by high eosinophilia. In this case, leukocytosis occurs in the blood, eosinophilia reaches 80-90%, and blast cells may be absent. High eosinophilia requires a bone marrow puncture, which in the case of leukemia reveals a high percentage of blast cells. In remission, eosinophilia disappears and reappears, sometimes as the first sign of relapse.

In children, enlarged submandibular lymph nodes are common due to chronic tonsillitis, and an enlarged spleen is a common reaction to infection. Leukemic infiltration of the lymph nodes gives them density (the nodes are usually painless); supraclavicular lymph nodes are often enlarged, rather than submandibular ones, as with tonsillitis. In doubtful cases, organ puncture is always indicated: this is especially true for the spleen, since it may have a local relapse with an almost complete content of blasts in the punctate. Timely diagnosis of acute lymphoblastic leukemia as a “rapid” leukemia has vital importance not only because of the rapidity of the process itself, but also because of the introduction of pathological cells into meninges.

According to the clinical picture, the ability to preserve normal hematopoietic germs, the frequency of the first remission, acute lymphoblastic leukemia in adults is similar to the childhood version.

In acute lymphoblastic leukemia, the spleen and lymph nodes enlarge for the most part simultaneously with the process in the bone marrow. Unlike acute myeloblastic leukemia, this increase in this leukemia is not new stage progression. Leukemic cells infiltrating the lymph nodes and spleen are usually sensitive to the same cytostatic drugs as cells in the bone marrow.

As a rule, improvement is achieved by using a complex of cytostatic agents. Further continuous treatment with cytostatic drugs maintains improvement for months or years. However, in adults, exacerbations of the disease often occur. Exacerbation can be either only local: the appearance of neuroleukemia, infiltration of nerve roots or infiltration of the testicle, leukemic episcleritis (inflammation outer shell eyeball), or bone marrow. Local exacerbation determined by spinal puncture, or patients experience pain caused by infiltration of the roots. A bone marrow exacerbation may not be accompanied by the release of blast cells into the blood, so the doctor performs a thoracic puncture regularly: monthly in the first year of improvement, and then once every 3 months. In addition, a thoracic puncture is performed when blasts appear in the blood and cytopenia does not depend on cytostatics.

The absolute indication for bone marrow examination should be clear Clinical signs exacerbations: enlarged lymph nodes, bone pain, radicular syndrome, low-grade fever, just unmotivated deterioration general condition. Signs of the disease during an exacerbation of acute lymphoblastic leukemia are stronger than during its first attack. Each subsequent exacerbation develops more malignantly than the previous ones and has a worse prognosis.

Metastasis of the process to the testicles and meninges, most common in childhood acute lymphoblastic leukemia, represents a new stage of tumor progression.

process of mediastinal nodes, early damage to the central nervous system and age younger than 1 year and older than 10 years.

Treatment of Acute lymphoblastic leukemia

The rate of improvement in children with this form of leukemia is 94%, in people over 15 years old - about 80%. The recovery rate in children is more than 50%. Prognostically unfavorable factors that affect the life expectancy of patients with acute lymphoblastic leukemia are the prevalence of the process at the time of diagnosis, leukocytosis above 15 H 103 in 1 μl, enlarged spleen, involvement of mediastinal nodes in the process, early damage to the central nervous system and age younger than 1 year and over 10 years old.

Immediate treatment with cytostatic drugs is carried out and only according to special programs.

The goal of treatment of acute leukemia is to achieve and maximize prolongation of improvement or recovery.

Acute lymphoblastic and undifferentiated leukemia in children. Treatment is carried out according to programs (developed by various authors), which allow more than 50% of children to maintain improvement for longer than 5 years.

Improvement is achieved in 4-6 weeks using one of 3 schemes; it should be noted that these schemes were introduced back in 1980-1990. and have not yet lost their relevance.

Vincristine 1.4 mg/m2 intravenously once every 7 days, prednisolone 40 mg/m2 per day (in regimens designed for 4-6 weeks, prednisolone is discontinued within 6-8 days).

Vincristine 1.4 mg/m2 intravenously once every 7 days, prednisolone 40 mg/m2 per day, rubomycin 60 mg/m2 2 days in a row on the 2nd week of therapy (on the 10th and 11th days of the course ).

Vincristine 1.4 mg/m2 1 time every 7 days intravenously, prednisolone 40 mg/m2 per day, L-asparaginase for 10 days 100 units/kg per day intravenously after 4-6 weeks of use of vincristine and prednisolone (if no full effect).

If treatment according to regimen 1 is ineffective for 4-6 weeks (in persons under 10 years of age), treatment according to regimen 2 or 3 is prescribed.

If there is no effect from the treatment regimens, the doctor prescribes combinations with Oncovin or Vinblastine.

Reinforcing courses are carried out 1-3 times, depending on the significance of the violation of the treatment conditions during the period of improvement, the length of this period, the prevalence of the leukemic process at the beginning of treatment, and the completeness of the improvement obtained. If the doctor discovers the spleen deep in the hypochondrium, this may serve as a basis for repeating the course of consolidation treatment. If the spleen is enlarged, the doctor punctures it and, if it has a lymphocytic composition, prescribes treatment aimed at maintaining the improvement.

Immediately after the diagnosis is made, a spinal puncture is performed with the introduction of methotrexate into the spinal canal at a dose of 12.5 mg/m2; during improvement and the course that consolidates the improvement, repeat regularly once every 2 weeks spinal taps with the introduction of methotrexate at a dose of 12.5 mg/m2. If any number of blast cells is detected in cerebrospinal fluid treatment for neuroleukemia is started, prophylactic irradiation of the head is canceled.

Achievement of improvement is necessarily confirmed by a control bone marrow puncture; first after diagnostic puncture bone marrow during the period of improvement is performed 7 days after the start of treatment (a decrease in blastosis in this punctate by 50% of the original or more means a good prognosis), then 4 weeks from the start of treatment.

The proliferative activity of leukemic cells increases sharply after a period of improvement, as after any cytostatic course. In this regard, immediately after improvement is achieved, the doctor prescribes maintenance treatment.

In combination, during the period of maintaining improvement, the dose of cytostatic drugs, excluding vincristine and prednisolone, is halved.

Development of polyneuritis (decreased tendon reflexes, muscle tone, numbness in the fingers and toes and the subsequent development of paresis of the limbs with muscle atrophy), caused by toxic effect vincristine requires reducing the dose of this drug by half, and if the changes become more pronounced or worsening, replacing it with vinblastine (a few weeks after stopping the drug, polyneuritis goes away). Treatment with cytostatic drugs is canceled when the level of leukocytes is below 1 H 103 (1000) in 1 μl, ulcerative stomatitis, diarrhea, severe vomiting, high fever lasting more than 2 days.

Prevention of neuroleukemia in acute lymphoblastic and undifferentiated leukemia in children is carried out with a cytologically normal composition of the cerebrospinal fluid (no blast cells, cytosis less than 10 in 1 μl) from the first week of improvement.

The first prophylaxis regimen: irradiation of the head in a total dose of 24 Gy and in parallel 5 injections of methotrexate endolumbarally. Prevention can be carried out mainly on an outpatient basis.

Prevention of acute lymphoblastic leukemia

Prevention

A dose of 24 Gy per head is given for 3 weeks, 1.5 Gy per session from two lateral fields.

It is advisable to make one of the two injections of methotrexate, administered 2 times a week into the spinal canal, during irradiation of the head on Saturday, since on this day, as a rule, there is no radiation therapy, the other - on one of the first days of the week

after a head irradiation session; on the day of endolumbar administration of methotrexate, the patient remains in the hospital.

During the period of prevention of neuroleukemia using both irradiation and the administration of methotrexate and cytosar, patients receive 6-mercaptopurine orally daily at a dose of 25 mg/m2 and cyclophosphamide at a dose of 100 mg/m2 once a week.

After the end of neuroleukemia prophylaxis, a bone marrow puncture is performed, and if there are no signs of relapse, then maintenance therapy is started.

The second method of preventing neuroleukemia is the endolumbar administration of methotrexate and cytosar. The drugs are administered at intervals of 3-4 days, if tolerated poorly, once a week.

Both methods of preventing neuroleukemia are reliable and eliminate the need for maintenance intralumbar injections of methotrexate.

Continuous maintenance treatment during the period of improvement of acute lymphoblastic and undifferentiated leukemia in children is carried out on an outpatient basis for 5 years up to complete improvement. Treatment begins immediately after complete improvement is achieved or after courses that consolidate the achieved improvement.

Children receive continuous treatment with three drugs according to the following regimen: 6-mercaptopurine orally daily; methotrexate orally on the 6th day of the week; cyclophosphamide orally on the 7th day of the week; on these days, 6-mercaptopurine is not discontinued.

For the “risk group”, during the period of continuous maintenance treatment with three drugs, a course of SO AR is administered every 1.5-2 months. During this course, maintenance treatment with three drugs is canceled for a week after it, and then given in half doses for a week. After this, maintenance treatment is carried out in full doses.

Conditions for continuous treatment:

1) blood test with determination of platelets and reticulocytes once a week;

2) when the level of leukocytes decreases to 1 H 103 - 2 H 103 (1000-2000) in 1 μl, the doctor reduces the dose of cytostatic drugs by half, with a subsequent increase of more than 2.5 H 103 (2500) in 1 µl restores the previous dose;

3) treatment is interrupted at any stage when the level of leukocytes in the blood drops below 1000 in 1 μl, with a significant increase in temperature, stomatitis, diarrhea;

4) bone marrow puncture in the first year of improvement is performed once a month; in the 2-5th year of improvement - once every 3 months.

Treatment for acute lymphoblastic leukemia is enhanced when we're talking about about the process of T-cell nature. According to the program of American pediatric oncologists, to eliminate the manifestations of the disease in T-lymphoblastic acute leukemia, they begin with intravenous administration cyclophosphamide 1200 mg/m2 on the 1st day of treatment or between the 2nd and 5th days (with a leukocyte level of more than 5 H 104 (50,000) in 1 μl and significant organomegaly, requiring prior administration of allopurinol due to high levels uric acid in serum and the risk of developing uric acid diathesis). From the 3-4th day (or on the 1st day, if the administration of cyclophosphamide is delayed) weekly (once a week) under this program, as well as under the Aur program, vincristine is administered for 4 weeks, as well as

Prednisolone and rubomycin are used in doses and at times corresponding to regimen 2 for the treatment of acute lymphoblastic leukemia.

When remission is achieved, a course of its consolidation is carried out, including a 5-day continuous administration of Cytosar at 100 mg/(m2/day), taking thioguanine (or 6-mercaptopurine) at 50 mg/m2 every 12 hours for 5 days of Cytosar administration. Three courses of treatment with cytosar and thioguanine (6-mercaptopurine) are carried out with an interval of 14 days between courses.

Then, for 7-14 days, L-acnapaginase is administered intravenously at a dose of 200-300 IU/kg. In case of high white blood cell count and large mass tumor lymph nodes splenomegaly or hepatomegaly therapy should be carried out by prescribing to the patient a large number of liquids, alkaline drink, along with allopurinol for the prevention of uric acid diathesis.

If, in T-cell acute leukemia, enlarged lymph nodes are detected in the mediastinum that contract poorly from chemotherapy, then it is recommended local irradiation this area in a dose of 30 Gy; Local irradiation is also advisable if there is a significant increase in lymph nodes in any other area.

Treatment during the period of improvement in T-cell leukemia should be intensified: along with continuous therapy with 6-mercaptopurine, methotrexate, cyclophosphamide.

Which can be cured by consulting a doctor. To do this on time, you need to know everything about the disease down to the smallest detail.

What is acute lymphoblastic leukemia?

Acute lymphoblastic form is considered the most common among (approximately 80%).

Most often it affects boys under 6 years of age and young men; girls are affected several times less often.

Lymphoblastic leukemia affects the lymph nodes, bone marrow, thymus gland, and internal organs such as the spleen.

If the disease recurs after chemical therapy, the central nervous system is involved in its process.

In essence, lymphoblastic leukemia is a malignant disease of the hematopoietic system, which is characterized by the rapid and uncontrolled proliferation of lymphoblasts (immature lymphoid cells).

Causes

The exact causes of the disease have not been established. Currently, scientists can only identify provoking acute lymphoblastic leukemia factors, which include:

• Infectious diseases suffered during infancy.
• undergone chemotherapy, radiation therapy and treatment with ionizing radiation.
• A stillbirth that the mother had previously.
• Biological mutagens and their Negative influence on a pregnant woman.
• The baby's weight at birth is more than 4 kg.
• Genetic pathologies of the child, for example, Down syndrome, immunodeficiency and the like.
• Presence of malignant neoplasms in the family history.

Scientists believe that the disease develops due to a combination of several of these factors, as well as a predisposition to it.

Symptoms and signs

Lymphoblastic leukemia is characterized by the presence of certain syndromes:

• Intoxicating. Characterized by the usual signs of intoxication, that is, weakness, fever and rapid weight loss.
• Hyperplastic. Peripheral lymph nodes are enlarged; upon palpation, painful conglomerates of a dense texture are revealed. The joints hurt and swell, there is pain in the bones and bone fractures. If the infiltrate has penetrated the liver and spleen, severe pain appears in the abdominal area.
• Anemic. The skin becomes painfully pale, the mucous membranes of the mouth bleed, tachycardia and hemorrhagic syndrome appear (bloody vomiting, hemorrhages, ecchymosis).

Also observed individual symptoms. So, in boys, the testicles and kidneys may become enlarged and painful. Extensive hemorrhages in the retina and lung tissue. Appears respiratory failure, swelling optic nerve. Elements of an infiltrating nature may appear on the skin, which hurt on palpation.

Diagnostics

Diagnosis begins with collecting anamnesis, including family history, and analyzing symptoms. Afterwards tests and examinations are prescribed:

• General and biochemical tests blood. Show the level of hemoglobin, leukocytes and erythrocytes, the presence of thrombocytopenia, kidney and liver damage. Myelocytes and metamyelocytes are also found in the blood if there is no intermediate form of maturation.

The photo shows a blood picture in acute lymphoblastic leukemia

• Myelogram. It is carried out in 3 stages. First, a morphological cytology analysis is taken, showing hypercellularity of the bone marrow and infiltration of blast cells. Cytochemical analysis is then performed, followed by immunophenotyping to determine the cell type.
• Spinal puncture showing the presence of damage to the central nervous system by leukemia cells.
• Ultrasound. Determines the degree of enlargement of the lymph nodes and the size of the affected organs.
• Chest X-ray to see if the mediastinum is enlarged.

If a patient is prescribed chemotherapy, additional tests are performed, such as urine tests, ECG and echocardiography.

Treatment

The main thing is chemotherapy, which is divided into three stages:

• Induction. The duration takes approximately several weeks, and is carried out until a stable remission is achieved. It is chemotherapy using cytostatics. Medicines destroy leukemic cells and restore hematopoiesis. Vincristine, glucocorticosteroids, asparaginase, anthracycline and others can be used as drugs. Statistics show that in more than 85% of cases it is possible to achieve stable remission.
• Consolidation. Lasts up to several months. Aimed at destroying remaining pathogenic cells. The most commonly prescribed medications are methotrexate, 6-mercaptopurine, vincristine, prednisolone, cytarabine, asparaginase and other drugs. To increase the effect, it is recommended to administer the drugs intravenously.
• Maintenance therapy. The duration is several years. Therapy is aimed at maintaining remission and eliminating the risk of relapse. The patient is prescribed 6-mercaptopurine and methotrexate.

Depending on response to treatment, patient age and concomitant diseases Other medications may be prescribed. For example, taking multivitamins or antibiotics. IN in rare cases requires a bone marrow transplant.

The incidence of leukemia is more than 4 cases per 100,000 people. Most of them are hemoblastoses that develop from immature lymphocytes. There is no doubt that a diagnosis such as acute lymphoblastic leukemia causes people to panic. We will tell you why it develops and how it is treated.

What kind of disease is this?

Acute lymphoblastic leukemia (ALL) is an aggressive and dangerous disease hematopoietic system, which develops as a result of mutation of lymphocyte precursor cells (lymphoblasts). Pathologically altered cells begin to actively divide, giving rise to the cancer process.

Depending on the type of cells that mutate, the disease is classified into pre-pre-B leukemia, pre-B leukemia, B leukemia and T leukemia. B-types of leukemia account for 80-85% clinical cases lymphoblastic leukemia, T-type - up to 20%.

A large number of tumor clones displaces normal hematopoietic cells and provokes the development of anemia, thrombocytopenia and decreased immunity. The latter is due to a decrease in the number of mature leukocytes, which are able to protect the body from infections.

In the acute form of lymphoblastic leukemia, actively proliferating cells quickly spread throughout the body through the lymphatic and circulatory system, accumulating in the peripheral blood and internal organs. Blast damage to body systems negatively affects their functionality and further worsens the patient’s survival prognosis.

As for the prevalence of the disease, acute lymphoblastic leukemia accounts for up to 80% of all diseases of the hematopoietic system in children, which absolute value is at least 3 patients per 100,000 population per year. The first and largest peak of incidence occurs between the ages of 2 and 5 years (according to some sources - from 1 to 6), and boys get sick more often.

The second and third peaks of lower intensity occur in adolescence (13-15 years) and old age (over 60 years), respectively. The age distribution is largely determined by the activity of production of one or another type of lymphocytes, as well as the accumulation of weakened immune defenses of the body and the accumulation of chemical carcinogens in the body.

Reasons for the development of leukemia

The exact causes of the development of this disease have not yet been established, but hematologists talk about a number of risk factors.

One of the most common factors that provoke mutation is chromosomal pathologies: deletion, inversion, amplification and translocation of chromosome sections.

Genetic disorders affect hematopoietic system even during the period of fetal formation, however, the completion of the mutation usually requires exposure to additional factors external environment. In B-leukemia associated with a mutation in the MLL gene, the change in the blast ends before birth.

According to latest research In addition to Down syndrome, Fanconi anemia, neurofibromatosis type 1 and other chromosomal pathologies, some allelic variants of the IKZF1, ARID5B, CEBPE and CDKN2A genes are associated with an increased risk of developing blastic leukemia. Presence of leukemia close relative the patient increases the chance of developing the disease.

Risk factors for the development of childhood blastic leukemia also include a history of stillbirth in the pregnant woman, exposure to biological and chemical mutagens on her body, and a child weighing more than 4 kilograms at birth.

Exposure to radiation (multiple x-rays, living in a region with high level ionizing radiation, irradiation greatly increases the risk of developing ALL. The risk of developing lymphoblastic leukemia after courses of radiotherapy reaches 10%. Chemical mutagens (benzene) and cytostatic drugs also increase the risk of developing leukemia. The negative effects of smoking are also noted.

A pronounced risk factor is infectious diseases, causing a nonspecific immune response. The peak incidence of ALL (2-5 years) coincides with the time of maximum active production of B-lymphocytes (3 years) and active contact of the child’s immune system with the external environment.

According to the hypothesis of one of the researchers, the reason childhood leukemia Many nonspecific pathogens can become infectious. They provoke the occurrence of mutations in the blasts of children who are predisposed to them and have had little contact with various infectious agents in the first 2 years of life.

Symptoms of acute lymphoblastic leukemia

Acute leukemia develops very quickly: by the time symptoms appear and diagnosis accurate diagnosis the mass of pathological cells in the patient’s body can reach 4% of his total weight. This is due to the active division of the tumor clone over several months of the latent period of the disease.

ALL is characterized by the development of several symptom complexes:

• anemic;
• hemorrhagic;
• intoxication;
• infectious;
• hyperplastic (proliferative);
• osteoarticular.

Anemic syndrome is caused by a decrease in the production of red blood cells due to inhibition of the red germ of hematopoiesis by lymphoblastic cells. It is manifested by pallor of the skin and mucous membranes, shortness of breath, dizziness, fatigue, and compensating tachycardia.

The hemorrhagic symptom complex includes internal, gum and nasal bleeding, a large number of hematomas on the skin, hemorrhagic rash, hematuria, bloody vomiting and feces. These signs are caused by the displacement of megakaryoblasts (platelet precursor cells) and, accordingly, a decrease in the number of platelets (thrombocytopenia).

Intoxication syndrome is characterized by rapid weight loss, fever and weakness. Hyperthermia can be triggered by both intoxication and exposure to an infectious agent.

An infectious symptom complex appears when the body is infected with a viral or bacterial disease. The absence of mature leukocytes sharply reduces the activity of the immune response. As a result, the patient may experience worsening chronic infections(for example, pyelonephritis, herpes) or develop systemic diseases (pneumonia, fungal diseases and etc.).

Hyperplastic and osteoarticular syndromes are often combined under general concept, because they are caused by the same reason - infiltration of body tissues with atypical blasts. The proliferation of mutated immature lymphocytes provokes an increase in the size of the lymph nodes, liver, spleen, kidneys, testicles, infiltration into the skin (with the appearance of purplish-blue infiltrates - leukemides), pulmonary and bone tissue, as well as the meninges. Neuroleukemia (brain damage with the development of meningus-like symptoms) is typical for relapses, especially B-leukemia.

Diagnosis of the disease

In acute lymphoblastic leukemia, the symptoms that make up the characteristic clinical picture, appear at the second (advanced) stage of the disease. However, leukemia can be detected at an earlier – initial stage.

This is possible if the patient’s predisposition to the disease is known. Under this condition, even a blurry picture of a blood test without obvious clinical symptoms (slight thrombocytopenia, changes in the number of lymphocytes, rarely anemia) can be a reason for a more specific diagnosis. In other cases, leukemia is suspected when clinical symptoms or characteristic changes appear general analysis blood.

The CBC of a patient with acute lymphocytic leukemia reveals increase in ESR, a decrease in the number of red blood cells, platelets, hemoglobin (anemia of II-III degree) and hematocrit, an increase in the number of leukocytes with the so-called. “leukocyte failure” (decrease in the number of mature forms of white cells). Atypical blasts are detected in the peripheral bloodstream, and leukocytes have different sizes.

However, the results of this analysis are not the only and sufficient method for diagnosing acute lymphoblastic leukemia. The final diagnosis is made only after receiving a myelogram.

A myelogram is the result of an analysis of the number of cells present in the bone marrow tissue. The sample for testing is obtained by puncture of the sternum or ilium. To establish a diagnosis " acute leukemia“The number of blasts should be 20-30% of the total tissue or more. In lymphoblastic leukemia, the erythroid, neutrophil and platelet lineages of hematopoiesis are suppressed.

Clarification of blasts belonging to a specific germ is carried out using cytochemical analysis and immunophenotyping.

The results of a myelogram can clearly differentiate leukemia from other diseases.

In addition to the CBC and bone marrow puncture, the examination plan for a patient with leukemia necessarily includes such studies as:

• blood biochemistry analysis (study of the functioning of internal organs before and during therapy);
• Ultrasound (assessment of the structure and volume of visceral organs and lymph nodes);
• spinal puncture (exclusion of infiltration into the meninges);
• chest x-ray (examination of lymph nodes).

Therapy for lymphoblastic leukemia

Treatment of acute lymphoblastic leukemia necessarily includes intensive chemotherapy and supportive treatment methods (immunotherapy, antibiotics, transfusion of blood components, etc.).

Chemotherapy consists of two stages - intensive and maintenance. The first stage, in turn, is divided into two phases. In the first phase, high-dose chemotherapy is used to achieve remission, while the second phase is aimed at destroying residual tumor clones and finally stopping the proliferation of abnormal blasts.

At the first stage of treatment, polychemotherapy regimens with red, yellow and blue chemotherapy drugs, as well as hormonal immunosuppressants, are used. The administration of cytostatic drugs occurs mainly intravenously, and in case of high risk or the presence of neuroleukemia - intrathecally (using lumbar punctures) or into the ventricles of the brain using the Ommaya reservoir.

The duration of the first stage is about six months. The criterion for successful treatment and complete remission is the absence of immature lymphoblasts in the peripheral blood and a decrease in their proportion in the bone marrow tissue to 5% or less. The second stage lasts up to 3 years. All this time the patient takes supporting cytostatic drugs in tablets.

It should be noted that in acute lymphoblastic leukemia, treatment and survival largely depend on the presence of gene mutations that increase the likelihood of developing this disease. For example, with the “Philadelphia chromosome” (translocation 9 and 22 chr.), a mutant gene is formed that produces an oncogenic tyrosine kinase. The use of tyrosine kinase inhibitor drugs significantly lengthens the period of remission.

If there is a high risk of leukemia recurrence and there are no contraindications, a stem cell transplant from a donor (bone marrow transplant) is possible.

The impossibility of performing an operation in many cases is due not only to its complexity and lack of suitable donor, but also by disruptions in the functioning of the patient’s body and the inability to use high-dose therapy to prepare for transplantation.

Early detection of clinical symptoms, accurate diagnosis, correctly chosen course of treatment and careful patient care ensuring maximum sterility during chemotherapy significantly increase the chances of treatment success. Even with a disease as aggressive as acute lymphoblastic leukemia, much depends on the patient and his family.

– a malignant lesion of the hematopoietic system, accompanied by an uncontrolled increase in the number of lymphoblasts. Manifested by anemia, symptoms of intoxication, enlarged lymph nodes, liver and spleen, increased bleeding and respiratory disorders. Due to decreased immunity in acute lymphoblastic leukemia, infectious diseases often develop. Possible damage to the central nervous system. The diagnosis is made based on clinical symptoms and data laboratory research. Treatment – ​​chemotherapy, radiotherapy, bone marrow transplant.

Treatment and prognosis for acute lymphoblastic leukemia

The basis of therapy is chemotherapy. There are two stages of treatment for ALL: the intensive therapy stage and the maintenance therapy stage. The intensive therapy stage for acute lymphoblastic leukemia includes two phases and lasts about six months. In the first phase, intravenous chemotherapy is performed to achieve remission. The state of remission is indicated by normalization of hematopoiesis, the presence of no more than 5% of blasts in the bone marrow and the absence of blasts in the peripheral blood. In the second phase, measures are taken to prolong remission, slow down or stop the proliferation of cells of the malignant clone. Drugs are also administered intravenously.

The duration of the maintenance therapy phase for acute lymphoblastic leukemia is about 2 years. During this period, the patient is discharged to ambulatory treatment, drugs are prescribed for oral administration carry out regular examinations to monitor the condition of the bone marrow and peripheral blood. The treatment plan for acute lymphoblastic leukemia is drawn up individually, taking into account the level of risk in a particular patient. Along with chemotherapy, immunochemotherapy, radiotherapy and other techniques are used. In case of low effectiveness of treatment and a high risk of relapse, bone marrow transplantation is performed. Average five-year survival rate for B-cell acute lymphoblastic leukemia in childhood is 80-85%, in adults – 35-40%. With T-lymphoblastic leukemia, the prognosis is less favorable.