Acute myeloid leukemia is not a death sentence! Acute myeloid leukemia - symptoms and treatment Video: Acute myeloid leukemia.

The concept of acute myeloblastic (or myeloid) leukemia (abbreviated as AML) combines several types of cancer of the human hematopoietic system, in which the bone marrow becomes the focus of cancer.

Until today, oncohematologists do not have a single confidence in the exact causes of disruption of the hematopoietic system, so highlight special groups risk, and even more so to predict the likelihood of contracting myeloid leukemia, or blood cancer, is quite difficult. Science makes every effort to create effective methods diagnosis and treatment of AML, resulting in acute myeloid leukemia diagnosed on early stages, today has a favorable prognosis for survival.

How does myeloid leukemia develop?

If you imagine the role bone marrow, as a producer of the entire variety of blood cells, then myeloid leukemia will look like a kind of sabotage in this well-functioning production.

The fact is that disruption of the bone marrow in myeloid leukemia is accompanied by the release into the blood production system of a huge number of “immature” or underdeveloped white blood cells of myeloblasts - leukocytes that have not yet found their immune function, but at the same time began to multiply uncontrollably. As a result of such a mutation, the coordinated process of regular renewal of leukocytes in the blood is disrupted and the rapid displacement of full-fledged blood cells by abnormal precursor cells begins. In this case, not only leukocytes are displaced, but also red blood cells (erythrocytes) and platelets.

Types of myeloid leukemia

Due to the fact that the mutation of blood cells itself rarely develops in the body in a “pure” form, but is most often accompanied by other mutations of stem cells and other pathologies, there are many various forms and types of myeloid leukemia.

If until recently there were 8 main types, divided according to the origin of leukemic formations, today mutations that have occurred in cells at the genetic level are also taken into account. All these nuances affect the pathogenesis and prognosis of life expectancy in one form or another of the disease. In addition, determining the type of acute myeloid leukemia disease allows you to select an appropriate treatment regimen.

According to the FAB, variants of myelocytic lecosis are divided into the following subgroups:

Features of acute promyelocytic leukemia

APL, or APML, which stands for acute promyelocytic leukemia, is a subtype of myeloid leukemia M3 according to the FAB (French-American-British classification). In this malignant disease, abnormal numbers of promyelocytes, which are immature granulocytes, accumulate in the blood and bone marrow of patients.

Acute promyelocytic leukemia is defined by a typical chromosome translocation, leading to the formation of abnormal oncoproteins and uncontrolled division of mutated promyelocytes. It was discovered in the middle of the 20th century and for a long time was considered one of the fatal and ultra-acute forms of myeloid leukemia.

Currently, acute promyelocytic leukemia shows a unique response to treatments such as arsenic trioxide and trans-retinoic acid. Thanks to this, APL has become one of the most favorably predicted and curable subtypes of the disease acute myeloid leukemia.

The life expectancy forecast for this variant of AML in 70% of cases is 12 years without exacerbations.

Promyelocytic leukemia is diagnosed by bone marrow tests, blood tests and additional cytogenetic studies. The most accurate diagnostic picture can be obtained through PCR (polymerase chain reaction) research.

Characteristics of acute monoblastic leukemia

Acute monoblastic leukemia belongs to the interregional form of AML in accordance with the FAB classification - variant M5, which occurs in 2.6% of cases in children and in 6-8% of cases in adults (most often the elderly).

Indicators clinical picture practically no different from acute myeloid leukemia, although general symptoms are supplemented by more pronounced intoxication and high temperature bodies.

The disease is also characterized by signs of neutropenia with a predominance of necrotic changes in the nasopharyngeal mucosa and oral cavity, as well as inflammation of the tongue.

The main site of localization of the disease is the bone marrow, but an enlargement of the spleen and certain groups of lymph nodes is also observed. In the future, infiltration of the gums and tonsils, as well as metastasis of the tumor to the internal organs, may occur.

However, with timely analysis, detection malignant pathology and the use of modern treatment regimens, in 60% of cases a significant improvement in the patient’s condition is predicted.

Characteristics of eosinophilic leukemia

Acute eosinophilic leukemia develops as a result of malignant transformation of eosinophils and can occur against the background of adenocarcinoma of the thyroid gland, uterus, intestines, stomach, bronchial and nasopharyngeal cancer. This type of myeloid leukemia is similar to the reactive eosinophilia inherent in acute lymphoblastic leukemia (ALL) or myeloblastic leukemia. Therefore, to differentiate diagnostics, they resort to studies of specific cellular blood markers.

The most characteristic features of this subtype of myeloid leukemia are an increase in the number of eosinophils and basophils in a blood test, and an increase in the size of the liver and spleen.

Features of myelomonocytic leukemia

Of particular concern to modern oncohematologists is such a subgroup of AML as myelomonocytic leukemia, varieties of which most often affect children. Although among the elderly population the risk of contracting this type of myeloid leukemia is also high.

Myelocytic leukemia is characterized by an acute and chronic course, and one of the chronic forms is juvenile myelomonocytic leukemia, characteristic of children from the first year of life to 4 years. The peculiarity of this subtype is the frequency of its development in young patients and the greater predilection for the disease in boys.

Why does myeloid leukemia develop?

Despite the fact that the exact causes of leukemia have still not been established, in hematology there is a certain list of provoking factors that can have a destructive effect on the activity of the bone marrow:

• radiation exposure;
• unfavorable environmental living conditions;
• work in hazardous production;
• influence of carcinogens;
• side effects from chemotherapy for other forms of cancer;
• chromosomal pathologies – Fanconi anemia, Bloom and Down syndromes;
• the presence of pathologies such as Epstein-Barr virus, lymphotropic virus or HIV;
• other immunodeficiency conditions;
• bad habits, especially smoking, of parents of a sick child;
• hereditary factor.

How does myeloid leukemia manifest?

Due to the fact that the symptoms of myeloid leukemia vary depending on the forms and types of AML, the allocation of general clinical indicators to the category of symptoms is very arbitrary. As a rule, the first alarms are detected in the results of a blood test, which forces the doctor to prescribe additional methods diagnostics

AML in children

In the case of young children, who are most susceptible to juvenile myelomonocytic leukemia, the presence of the following symptoms should alert parents and force them to see a doctor:

1. If the child is not gaining weight well;
2. If there are delays or deviations in physical development;
3. Increased fatigue, weakness, pale skin due to iron deficiency anemia;
4. Presence of hyperthermia;
5. Frequent infections;
6. Enlarged liver and spleen;
7. Swelling of peripheral lymph nodes.

Of course, the presence of one or more of the above symptoms does not mean that the child is definitely developing juvenile myelocytic leukemia, because such indicators are characteristic of many other diseases. But, as you know, treatment of complex diseases is most effective in the early stages, so taking blood tests and undergoing other diagnostic procedures will not be superfluous.

AML in adults

• chronic fatigue, general weakness;
• loss of weight and appetite;
• tendency to internal hemorrhages, bruising, increased bleeding;
• increased bone fragility;
• frequent dizziness and chills;
• instability to infectious pathologies;
• nausea;
• constant pallor.

It is clear that these symptoms cannot serve as the only factor in determining AML, so you should not self-diagnose cancer.

Diagnostic procedures for AML

The first and fundamental diagnostic measure for verifying myeloid leukemia is a complete blood test. If a pathological proliferation of certain groups of blood cells is detected, a bone marrow biopsy is prescribed. To determine the distribution cancer cells used in the body:

• X-ray and ultrasound examinations;
• skeletal scintigraphy;
• computed and magnetic resonance imaging.

As a rule, all diagnostic procedures are carried out in hematology and oncology clinics, and when the diagnosis of AML is confirmed, a treatment plan is immediately drawn up. Since the pathogenesis (course) of different forms of the disease differs at the cellular and molecular level, the prognosis of the patient’s life expectancy depends entirely on the accuracy of the diagnosis and the adequacy of the chosen treatment method.

Therapeutic measures

Today, treatment of myeloid leukemia consists of 4 stages of therapeutic measures:

1. Induction with intensive use of chemotherapy designed to destroy as many myeloid cells as possible in the shortest possible time to achieve a remission period.
2. Consolidation with intensive care combination and additional chemotherapy doses to destroy remaining tumor cells and reduce the risk of the disease returning.
3. Treatment of the central nervous system, carried out to prevent leukemia cells from reaching the spinal cord and brain, to prevent metastasis. If leukemia cells fall into the central nervous system, a course of radiation therapy may be prescribed.
4. Long-term maintenance therapy prescribed for long period(a year or more) and carried out on an outpatient basis with the aim of destroying surviving cancer cells.

Side effects of chemotherapy

Despite the effectiveness of chemotherapy treatment, not every patient agrees to use high doses of chemotherapy, since this technique has a significant drawback - side complications.

Is it possible to defeat leukemia?

Today it is too early to talk about a complete victory over leukemia. But an increase in life expectancy after intensive therapy by at least 5-7 years is noted on average in 60% of patients. True, the forecasts for patients over 60 years of age do not rise above a 10% indicator. Therefore, you should not wait until you reach old age in order to take serious care of your own health. You need to undergo preventive examinations, monitor your diet and lifestyle, and donate blood and urine tests regularly.

Myeloblastic leukemia is often classified as a disease of the young. According to statistics, it is most often determined in people who have barely crossed the threshold of 30-40 years. If we talk about the prevalence of pathology, then such a disease occurs in 1 case per 100 thousand population. There is no dependence on gender or race.

What is the essence of the problem?

In the picture you can see what the blood composition is in a healthy person, and how it changes with leukemia:

Many people are naturally concerned about the question: what is it? Acute myeloid leukemia (or acute myelocytic leukemia) is an oncological pathology affecting the blood system, when there is an uncontrolled proliferation of leukocytes of an altered shape. In addition, the blood test will also contain problems such as a decrease in the number of red blood cells, platelets and white blood cells from the normal category.

Blood diseases are particularly dangerous for humans. And this is not without reason, because it is the blood that is in close contact with all organs and systems in the body, it is the blood that carries vital hemoglobin and oxygen. Therefore, it is extremely important that blood circulation is fully and correctly established. The cellular composition should remain within normal limits.

Acute myeloid leukemia (AML) is a condition in which immature blood cells called blasts change. At the same time, the body experiences a lack of mature cells. The modified blasts increase virtually exponentially.

It is important to understand here that the process of cell change is irreversible and is not controlled by any medications. Blast cell leukemia – severe and life-threatening disease.

Typically, all pathological processes in such a situation are localized in the bone marrow and peripheral system blood circulation Malignant cells actively suppress those that are not broken or spoiled and begin to infect literally everything in the body.

What is leukemia and can it be prevented? What is chemotherapy? Find out the answers to your questions by watching the following video:

Types of problem

Acute myeloblastic leukemia is a fairly broad group pathological changes in the human body. Thus, there are several options when classifying this pathology:

• M0 is a dangerous variety that is highly resistant to chemotherapy and has an extremely unfavorable prognosis for the patient’s life.
• M1 is a rapidly progressive type of myeloblastic leukemia with a high content of blast cells in the blood.
• M2 – the level of mature leukocytes is about 20%.
• M3 (promyelocytic leukemia) - characterized by an active accumulation of immature leukocytes in the bone marrow.
• M4 (myelomonocytic leukemia) – treated with chemotherapy and bone marrow transplant. It is more often diagnosed in children and has an unfavorable prognosis for life.
• M5 (monoblastic leukemia) – about 25% of blast cells are detected in the bone marrow;
• M6 (erythroid leukemia) is rare and has a poor prognosis.
• M7 (megakaryoblastic leukemia) is a pathology with injury to the myeloid lineage that develops in Down syndrome;
• M8 (basophilic leukemia) - diagnosed in children and adolescents. Together with myeloblastic cells, atypical basophils are detected.

More detailed classification leukemia is shown in the picture:

The choice of treatment tactics, life prognosis and the duration of remission intervals directly depend on the type of leukemia.

Reasons for the development of the problem

Leukemia, acute myeloid leukemia - all this is the same name for such a pathology. Naturally, many people are concerned about what leads to the development of such a problem. But, as is the case with other types of oncology, doctors cannot name the catalyst for changes in blood cells with 100% certainty. However, it is possible to identify predisposing factors.

Today, the main reason for the development of pathology is called chromosomal disorders. Usually they mean the situation called the “Philadelphia chromosome”. This is a condition when, due to a disorder, entire sections of chromosomes begin to change places, forming completely new structure DNA molecules. Then copies of such malignant cells are quickly formed, which leads to the spread of pathology.

According to doctors, this situation can be caused by:

• Exposure to radiation. For example, those at risk are those who are in production areas with a large amount of radiation, rescuers working at the site of removing rubble, as was the case at Chernobyl nuclear power plant, patients who have previously undergone radiation for the treatment of another type of oncology.
• Some viral diseases.
• Electromagnetic radiation.
• Impact of a number medicines. Usually in this case they mean anti-cancer therapy due to its increased toxicity to the body.
• Heredity.

Those who are at risk should be screened regularly.

Symptoms of pathology

AML in adults, as in children, is characterized by certain symptoms and signs. Cells multiply and change at an uncontrollable speed, so signs of the disease appear quite quickly, and a person cannot ignore them - they are too bright.

Symptoms of this pathology include:

• Pallor skin– this symptom is often called one of the first and characteristic, because it accompanies all hematopoietic pathologies.
• Signs of anemia.
• Uncontrolled bleeding that is sometimes difficult to stop.
• Appearance low-grade fever– it can fluctuate between 37.1-38 degrees, sweating appears during night sleep.
• The appearance of a rash on the skin - it consists of small red spots that do not itch.
• The appearance of shortness of breath - and it is typical even for small physical activities.
• The appearance of a feeling of pain in the bones, especially intensified while walking, but the pain is not severe, so people can get used to it.
• The appearance of swelling in the gums, the development of bleeding and gingivitis.
• The appearance of hematomas - such spots are red- blue can appear in any part of the body.
• Decreased immunity and frequent infectious diseases.
• Sudden weight loss.

What are the defined norms of blood parameters for a healthy person, see the picture:

The second part of the table in the following picture:

The age of the patient is not important. Everyone has these signs if present of this disease will appear. If intoxication affects the brain, neurological symptoms appear: headaches, epiactivity, vomiting, ICP, hearing and vision impairment.

Children may exhibit the following symptoms:

• Memory impairment;
• Apathy and reluctance to play;
• Stomach ache;
• Gait changes.

How to diagnose?

Since AML always manifests itself sharply, it will not be possible to delay going to the doctor. At the appointment, the specialist will offer a whole range of measures that will allow you to make an accurate diagnosis and select the optimal treatment methods. First, inspection and questioning. Then the following diagnostic procedures are suggested:

• General blood test. They will pay here special attention on the number of leukocytes in the blood. In the blood of patients, the number of immature white cells increases, and a change in the number of platelets is noted. A blood test is one of the main ones on this list.
• Biochemical blood test. Such an analysis for leukemia will show high content vitamin B12, as well as uric acid and a number of enzymes.
• Bone marrow biopsy.
• Cytochemistry – blood and bone marrow samples are taken for study.
• Ultrasound - this method helps to determine the increase in the size of the liver and spleen (these organs usually increase with this pathology).
• Genetics research.

Based on the data obtained, the doctor will be able to make an accurate diagnosis and determine the direction of therapy.

How to treat?

Medical procedures include the following options:

• Chemotherapy;
• Radiation therapy;
• If necessary, a bone marrow transplant, as well as stem cells (material is taken from a donor);
• Leukapheresis is a procedure where changed cells are removed;
• Splenectomy.

However, in most cases, curing the disease is difficult and even impossible. Therefore, therapy is more symptomatic. With its help, they alleviate the patient’s condition and support his vital functions.

Myeloblastic leukemia - a malignant blood disease characterized by the uncontrolled growth of immature blood cells (myeloblasts). Accumulating in the bone marrow, peripheral blood and internal organs, they cause severe dysfunction of all body systems.

Laboratory indicators

General blood test

• E red blood cells: reduced
• Reticulocytes: reduced
• Leukocytes: varies significantly from 0.1 10 9 /l to 100.0 10 9 /l,
  1. in 38% of patients leukocytes are normal or reduced
  2. in 44% increased to 15-20 10 9 /l
  3. 18% exceed 50.0 10 9 /l

• Platelets are reduced, less than 130.0 10 9 /l

• There are no eosinophils in the peripheral blood

• There are no basophils in the peripheral blood
• ESR increased, over 15 mm/hour
• Myeloblasts in the blood are defined as immature cells (more than 20%)

Bone marrow analysis

• The content of immature cells (myeloblastic cells) is more than 20%.
• The number of other bone marrow growth cells is reduced

Other studies:
Cytochemical reactions to determine the type of leukemia.

• Myeloid leukemia is characterized by positive reactions to lipids and to a specific enzyme (peroxidase). The reaction to glycogen is negative.

Immunological reactions (IR).
IR helps to most accurately determine the type of leukemia. Specific markers (antibodies) are used to structures on blood cells (antigens) characteristic specifically for certain type leukemia.

Symptoms

1. Symptoms of intoxication:

• Temperature 38 - 40 °C
• Headache
• Excessive sweating
• Muscle pain

1. Symptoms of increased bleeding due to decreased platelets:

• Pinpoint subcutaneous hemorrhages
• Easily bruise
• Bleeding gums
• Nosebleeds, gastrointestinal, uterine

1. Symptoms caused by decreased red blood cells (anemia)

• Pale skin
• General weakness
• Lack of air during physical activity
• Dizziness
• Rapid heartbeat

1. Symptoms associated with excessive proliferation of myeloblastic cells:

• Enlarged lymph nodes(rarely)
• Enlarged liver and spleen (not in all cases)
• Joint pain (rare)
• Red-blue spots on the skin (leukemids) - rare
• Nervous system lesions (rare)
• Rapid depletion vital important organs(heart, kidneys, lungs)

1. Symptoms of infectious complications due to a decrease in the number of leukocytes:

• Bacterial and fungal infections(necrotizing stomatitis, tonsillitis, bronchitis, pneumonia, paraproctitis, etc.).

Cause of myeloblastic leukemia

Under the influence of ionizing radiation, viruses or chemicals, a mutation occurs in the mother blood cell. Its uncontrolled division leads to the spread of tumor cells throughout the body.

How to treat?

Main directions:

• Chemotherapy
• Transfusion of blood components
• Antibiotic therapy
• Bone marrow transplant

Chemotherapy is performed according to special programs:

1. Remission activation stage.

• Scheme “7+3”, Cytosar 100 mg/m², 2 times a day, 7 days; Rubomycin 45/m², 1 time per day , 3 days.
• Scheme “5+2”, Cytosar - 5 days, Rubomycin - 2 days. (Scheme for persons over 60 years of age)

The scheme is repeated 2-3 times until incomplete remission is achieved (disappearance of symptoms caused by excessive proliferation of blast cells, normalization of blood and bone marrow tests, myeloblast cells in the bone marrow test no more than 20%).

1. Stage of strengthening remission.

• The “7+3” or “5+2” schemes are prescribed, 2-3 courses each.

1. Prevention of complications from the nervous system (non-leukemia).

Carried out at the stage of activation of remission, drugs (Dexamethasone, Methotrexate, Cytosar) are administered under the membranes spinal cord(intrathecal).

1. Remission maintenance stage

Monthly Cytosar 5 days 100 mg 2 times a day, together with Thioganin 10 mg/m², every 12 hours. Repeat this scheme for 5 years.
Transfusion of blood components:

• Cryoplasma
• Platelet concentrate
• Red blood cell mass

Goal: to restore the deficiency shaped elements blood (erythrocytes, platelets, etc.).
Antibiotic therapy
Goal: to prevent infectious complications associated with taking chemotherapy drugs that reduce the number of immune cells(leukocytes). Treatment options:

• Tsiprinol 1 g/day in combination with Diflucan 400 mg daily
• Biseptol 6 tablets a day in combination with nystatin 4-6 million units/day

Alternative option The treatment for myeloblastic leukemia is a bone marrow transplant (usually from a brother or sister).

– a malignant disease of the blood system, accompanied by the uncontrolled proliferation of altered leukocytes, a decrease in the number of red blood cells, platelets and normal leukocytes. It is manifested by an increased tendency to develop infections, fever, fatigue, weight loss, anemia, bleeding, the formation of petechiae and hematomas, pain in bones and joints. Sometimes skin changes and swelling of the gums are detected. The diagnosis is made based on clinical symptoms and data laboratory research. Treatment is chemotherapy, bone marrow transplantation.

ICD-10

C92.0

General information

Acute myeloid leukemia (AML) is a malignant lesion of the myeloid blood lineage. Uncontrolled proliferation of leukemia cells in the bone marrow leads to the suppression of other blood sprouts. As a result, the number of normal cells in the peripheral blood decreases, anemia and thrombocytopenia occur. Acute myeloid leukemia is the most common acute leukemia in adults. The likelihood of developing the disease increases sharply after age 50. The average age of patients is 63 years. Men and women of young and middle age suffer equally often. In senior age group There is a predominance of males. The prognosis depends on the type of acute myeloid leukemia, with five-year survival rates ranging from 15 to 70%. Treatment is carried out by specialists in the field of oncology and hematology.

Causes of acute myeloid leukemia

The direct cause of the development of AML is various chromosomal abnormalities. Risk factors contributing to the development of such disorders include unfavorable heredity, ionizing radiation, contact with certain toxic substances, taking a number of medications, smoking and blood diseases. The likelihood of developing acute myeloid leukemia increases with Bloom's syndrome (short height, high voice, characteristic features faces and diverse skin manifestations, including hypo- or hyperpigmentation, skin rash, ichthyosis, hypertrichosis) and Fanconi anemia (short stature, pigmentation defects, neurological disorders, abnormalities of the skeleton, heart, kidneys and genitals).

Acute myeloid leukemia quite often develops in patients with Down syndrome. There is also a hereditary predisposition in the absence of genetic diseases. With AML in close relatives, the likelihood of developing the disease increases 5 times compared to the population average. Most high level correlations are detected in identical twins. If acute myeloid leukemia is diagnosed in one twin, the risk in the other is 25%. One of the most important factors Blood diseases that provoke AML are blood diseases. Chronic myeloid leukemia in 80% of cases transforms into acute form diseases. In addition, AML is often the outcome of myelodysplastic syndrome.

Ionizing radiation causes acute myeloid leukemia when the dose exceeds 1 Gy. The incidence increases in proportion to the radiation dose. In practice, staying in areas of atomic explosions and accidents at nuclear power plants, working with radiation sources without appropriate protective equipment, and radiotherapy used in the treatment of certain cancers are important. The cause of the development of acute myeloid leukemia upon contact with toxic substances is bone marrow aplasia as a result of mutations and damage to stem cells. The negative effects of toluene and benzene have been proven. Typically, AML and other acute leukemias are diagnosed 1-5 years after exposure to the mutagen.

Among the drugs that can provoke acute myeloid leukemia, experts name some drugs for chemotherapy, including DNA topoisomerase II inhibitors (teniposide, etoposide, doxorubicin and other anthracyclines) and alkylating agents (thiophosphamide, embiquin, cyclophosphamide, chlorambucil, carmustine, busulfan). AML can also occur after taking chloramphenicol, phenylbutazone and arsenic drugs. The share of drug-induced acute myeloid leukemia is 10-20% of the total number of cases of the disease. Smoking not only increases the likelihood of developing AML, but also worsens the prognosis. The average five-year survival rate and duration of complete remissions are lower for smokers than for non-smokers.

Classification of acute myeloid leukemia

The WHO classification of acute myeloid leukemia is very complex and includes several dozen types of the disease, divided into the following groups:

• AML with typical genetic changes.
• AML with changes due to dysplasia.
• Secondary acute myeloid leukemia resulting from treatment of other diseases.
• Diseases with myeloid lineage proliferation in Down syndrome.
• Myeloid sarcoma.
• Blastic plasmacytoid dendritic cell tumor.
• Other types of acute myeloid leukemia.

Treatment tactics, prognosis and duration of remissions for different types AMLs can vary significantly.

Symptoms of acute myeloid leukemia

The clinical picture includes toxic, hemorrhagic, anemic syndromes and the syndrome of infectious complications. In the early stages, the manifestations of acute myeloid leukemia are nonspecific. There is an increase in temperature without signs of catarrhal inflammation, weakness, fatigue, loss of weight and appetite. With anemia, dizziness, fainting and pallor of the skin occur. With thrombocytopenia, increased bleeding and petechial hemorrhages are observed. Possible formation of hematomas with minor bruises. With leukopenia, infectious complications arise: frequent suppuration of wounds and scratches, persistent repeated inflammation of the nasopharynx, etc.

Induction programs can achieve remission in 50-70% of patients with acute myeloid leukemia. However, without further consolidation, most patients relapse, so the second stage of treatment is considered an obligatory part of therapy. The consolidation treatment plan for acute myeloid leukemia is drawn up individually and includes 3-5 courses of chemotherapy. At high risk relapses and already developed relapses, bone marrow transplantation is indicated. Other treatments for recurrent AML are still under development. clinical trials.

Prognosis of acute myeloid leukemia

The prognosis is determined by the type of acute myeloid leukemia, the age of the patient, and the presence or absence of a history of myelodysplastic syndrome. Average five-year survival rate different forms AML ranges from 15 to 70%, the probability of relapse is from 33 to 78%. Older people have a worse prognosis than younger people, which is explained by the presence of concomitant somatic diseases, which are a contraindication for intensive chemotherapy. With myelodysplastic syndrome, the prognosis is worse than with primary acute myeloid leukemia and AML that arose during pharmacotherapy for other cancers.

Acute myeloid leukemia is a malignant disease of the hematopoietic system that usually occurs in children under 15 or elderly over 60 years of age. Here we talk about the reasons for the development of this disease, its symptoms, methods and prospects for treatment.

What is myeloid leukemia?

Acute myeloid leukemia (leukemia, leukemia) develops as a result of mutation of the hematopoietic cells of the myeloid lineage of the bone marrow.

Unlike the lymphoblastic type of leukemia, with myeloid leukemia, not only leukocyte precursors can mutate, but also blast cells, which during normal development develop into erythrocytes and platelets. In most cases of AML, uncontrolled proliferation of white blood cells is observed, which determines the second name of the disease (bleeding).

Despite the fact that any of the hematopoietic cells can undergo mutation, the symptoms of the disease are almost the same. This is due common mechanism development of leukemia.

Uncontrolled proliferating immature blasts actively displace functional cells of other hematopoietic lineages, causing (depending on the type of leukemia) thrombocytopenia, anemia and decreased immunity. However, the mutated progenitor cells themselves are not able to perform their functions due to their low degree of maturity.

This leads to the fact that with any type of leukemia there is a decrease in the number of red blood cells, platelets and functional immune cells - leukocytes.

Acute myeloid leukemia usually develops in older patients: the risk of its manifestation increases sharply in people over 50 years of age, and middle age The patient with AML is 63 years old. In the older and younger (up to 15 years) age groups of morbidity, men predominate; in young and middle age, there are no differences in risks by gender.

Causes of development of myeloid leukemia

The exact reasons that are guaranteed to lead to the development of myeloid leukemia have not been established. However, hematologists and oncologists identify a number of factors that significantly increase the risk of this blood disease. These include:

• pre-leukemic pathologies of hematopoiesis (myelodysplastic and myeloproliferative syndromes against the background of characteristic cytogenetic changes, PNH, etc.);
• use of anticancer drugs for other cancer(the use of anthracyclines and alkylating substances carries high risks);
• prolonged contact with chemical carcinogens (phenylfutazone, arsenic compounds, chloramphenicol, toluene, benzene, etc.);
• ionizing radiation;
• hereditary predisposition (presence of the disease in close relatives) and the presence of chromosomal pathologies associated with an increased risk of AML (Down syndrome, balanced and unbalanced abnormalities in chromosome regions, characteristic gene mutations).

Acute myeloid leukemia of drug origin accounts for up to 20% of all clinical cases mutations in myeloid lineage cells. As a rule, this pathology occurs within 3-5 years after the end of chemotherapy for another cancer.

The effects of chemical carcinogens appear within 1-5 years after contact with them. Experts point out that this contact with harmful reagents increases negative impact other risk factors, but is rarely the sole cause of AML development.

According to some studies, smoking also increases the chances of developing acute leukemia. Five-year survival prognoses and the average duration of complete remissions in smoking patients are worse than in non-smoking patients.

Symptoms of acute myeloid leukemia

The clinical picture of myeloid leukemia is similar to the symptoms of lymphoblastic leukemia. When the hematopoietic cells of the myeloid lineage are mutated, anemic, hemorrhagic, intoxication and infectious syndromes are also observed.

However, the proliferative syndrome has a number of features: for example, with AML there are no pronounced changes in the volume of peripheral lymph nodes, liver and spleen. In some cases, an increase in supraclavicular and cervical lymph nodes is detected, while others remain small and mobile.

Infiltration of the body by immature blood cells manifests itself in bones and joints. Patients complain of pain in the spine and bones lower limbs, their movements are limited and their gait changes. Often the spread of leukemia cells is manifested by exophthalmos (protrusion of eyeball), swelling and inflammation of the gums without compromising their integrity, enlarged tonsils.

Anemia, caused by a lack of red blood cells, is manifested by the patient's skin and mucous membranes becoming pale (sometimes they may take on an earthy tint), constant weakness, dizziness, increased heart rate and shortness of breath.

Thrombocytopenia provokes increased bleeding, the appearance of a hemorrhagic rash, and large hematomas. Hemorrhages can be observed both externally (from the gums, nose and skin lesions) and internally. Internal bleeding is detected by blood in the urine (hematuria), vomit and stool.

Despite the small number of platelets, blood viscosity can remain and even increase due to the rapid proliferation of mutant cells. High blood viscosity can provoke heart attacks and hemorrhages in internal organs.

Due to non-functionality or low number of leukocytes, the patient's immunity is reduced. This is manifested by frequent viral and bacterial infections, suppuration of small wounds, fever. Fever and lethargy can also be caused by intoxication of the body with blast cells. In almost 100% of cases of AML, patients lose their appetite and experience rapid weight loss.

On initial stage development of the disease, AML can be asymptomatic or nonspecific. At the second – advanced – stage, a pronounced toxic syndrome is observed, first of all. Relapses develop in a similar sequence.

Additional signs of myeloid leukemia

If immature cells enter the central nervous system, the patient may have a headache, vomiting, impaired vision and activity facial nerves. These manifestations are called leukemic meningitis.

With hyperleukocytosis and infiltration of blasts into the lung tissue (leukemic pneumonia), respiratory function. According to statistics, this manifestation of myeloid leukemia is observed in every fourth patient.

Myeloid sarcoma, which accounts for up to 10% of all clinical cases of AML, is characterized by skin manifestations - dense tumor-like leukemides of a greenish, pink, gray, white or brown hue, often with inflammation of the skin around them.

Men with AML may experience enlargement of one or both testicles.

Diagnosis of the disease

Diagnosis of myeloid leukemia is usually carried out at the second stage of the disease, when clinical symptoms appear. The hematologist collects the patient’s medical history and monitors the dynamics of changes in the composition general analysis blood and performs a bone marrow puncture (the sternum or iliac crest is designated as the site for tissue collection).

Characteristic changes in the CBC are considered to be an increase in the erythrocyte sedimentation rate and the total number of leukocytes (in 62% of cases) against the background of a decrease in the concentration of mature white cells, hemoglobin, hematocrit, reticulocytes, platelets and the absence of eosinophils and basophils (with a mutation of the neutrophil sprout).

The final diagnosis is made based on the results of a myelogram - a quantitative and qualitative analysis of bone marrow tissue. In myeloid leukemia, cytochemical analysis reveals positive reaction for myeloperoxidase and lipids, but negative for glycogen. The chemical method allows you to quickly differentiate AML and lymphoblastic leukemia. The type of mutant cell is clarified using immunophenotyping. In AML, the number of immature cells in the bone marrow tissue is at least 20%.

To identify characteristic mutations that can affect the survival prognosis and require a special approach to treatment, a cytogenetic study of the biomaterial is carried out.

Due to the fact that the proliferation of immature leukocytes (and other cells of the myeloid lineage) negatively affects the entire body, simultaneously with the diagnosis of leukemia, a study of the condition is carried out internal organs, bone and lymphatic system, meninges. For this, ultrasound, radiography, biochemical blood tests and lumbar puncture are used.

Treatment and prognosis for acute myeloid leukemia

Despite the danger of the disease, acute myeloid leukemia can be cured. With favorable prognosis, no contraindications to bone marrow transplantation and timely initiation of chemotherapy, the risks of disease relapse are reduced to minimal.

The classic treatment for AML is a combination of polychemotherapy and stem cell transplantation. The course consists of three stages:

1. Induction (activation). The goal of the first stage is to achieve complete remission of the disease (reducing the number of immature cells in the bone marrow tissue to 5% or less). Therapy is carried out with cytarabine (Cytosar) and anthracyclines (Idarubicin, Rubomycin, etc.) according to the “5+2” or “7+3” scheme (5-7 days of cytarabine administration, in the first 2-3 days together with anthracyclines). The induction stage can be supplemented with other drugs depending on the type of leukemia - for example, PTRK for promyelocytic leukemia. Complete remission occurs in 50-70% of clinical cases of AML.
2. Consolidation. Serves for the final destruction of abnormal cells and restoration of hematopoiesis. At low risks of relapse, patients are prescribed 3-5 chemotherapy courses and immunotherapy; at high risks, a bone marrow transplant from a suitable donor is prescribed. High risks of relapse are usually associated with cytogenetic changes.
3. Maintenance therapy. Helps increase the duration of remission and includes 5-day monthly courses of chemotherapy drugs in tablets for 2-5 years.

In the first two stages, blasts are completely destroyed, so to exclude side effects treatment, the patient is given an infusion of blood components, antibacterial and detoxification therapy. The actual absence of one's own immunity necessitates maintaining sterile conditions for the patient.

In case of severe dysfunction of internal organs, a high risk of infectious complications and the patient’s advanced age, palliative therapy is used instead of a high-dose course.

The average effectiveness of AML therapy is 20-45% in terms of five-year survival. The prognosis is less favorable than for lymphoblastic leukemia due to age and the frequency of somatic disorders in patients with myeloid leukemia.

Comprehensive diagnosis is an important step in the treatment of myeloid leukemia. Taking into account the functionality of internal organs, cytogenetic characteristics and the type of abnormal cells when selecting a treatment course, the doctor can significantly increase the chances of achieving stable remission and long-term survival of the patient.