Chronic myeloid leukemia symptoms of the disease. How many people live with chronic myeloid leukemia, and how the stage of the course of the disease affects life expectancy. Removal of the spleen splenectomy

Blast crisis in chronic myelogenous leukemia

Blast crisis in patients with chronic myelogenous leukemia occurs immediately after the advanced stage, and its main feature is a significant increase in the number of blasts in the bone marrow (above 30%). Accompanied by severe pain in the bones, body weight continues to decline, and fever and discomfort in the spleen region persist. The patient is susceptible to all kinds of infectious diseases due to a decrease in immunity, bruises and bruises appear on his body, which indicates a decrease in the number of platelets.

Blast crisis in chronic myeloid leukemia is divided into several types: lymphoblastic (lymphoid) and myeloid, which occur respectively in 65 and 25% of cases. Another 10% falls on the rarest variety - erythroblast crisis.

Chronic myeloid leukemia- a tumor disease of the blood. It is characterized by uncontrolled growth and reproduction of all blood germ cells, while young malignant cells are able to mature to mature forms.

Chronic myeloid leukemia (synonymous with chronic myeloid leukemia) - a tumor disease of the blood. Its development is associated with changes in one of the chromosomes and the appearance chimeric (“cross-linked” from different fragments) of a gene that disrupts hematopoiesis in the red bone marrow.

During chronic myeloid leukemia, the content of a special type of leukocytes increases in the blood - granulocytes . They are formed in the red bone marrow in large quantities and enter the blood without having time to fully mature. At the same time, the content of all other types of leukocytes decreases.

Some facts about the prevalence of chronic myelogenous leukemia:

• Every fifth tumor disease of the blood is chronic myelogenous leukemia.
• Among all blood tumors, chronic myelogenous leukemia ranks 3rd in North America and Europe, and 2nd in Japan.
• Globally, chronic myelogenous leukemia occurs in 1 in 100,000 people every year.
• Over the past 50 years, the prevalence of the disease has not changed.
• Most often, the disease is detected in people aged 30-40 years.
• Men and women get sick with about the same frequency.

Causes of Chronic Myeloid Leukemia

The following factors are believed to be relevant:

As a result of breakdowns in chromosomes, a DNA molecule with a new structure appears in the cells of the red bone marrow. A clone of malignant cells is formed, which gradually crowd out all the others and occupy the main part of the red bone marrow. The vicious gene provides three main effects:

• Cells multiply uncontrollably, like cancer cells.
• For these cells, the natural mechanisms of death cease to work.

Phases of chronic myeloid leukemia

• chronic phase. The majority of patients who visit a doctor (about 85%) are in this phase. The average duration is 3-4 years (depending on how timely and correctly the treatment is started). This is the stage of relative stability. The patient is concerned about minimal symptoms to which he may not pay attention. Sometimes doctors discover chronic phase myelogenous leukemia by accident during a complete blood count.
• Acceleration phase. During this phase, the pathological process is activated. The number of immature white blood cells in the blood begins to increase rapidly. The acceleration phase is, as it were, a transitional phase from chronic to the last, third.
• Terminal phase. final stage of the disease. Occurs with an increase in changes in chromosomes. The red bone marrow is almost completely replaced by malignant cells. During the terminal stage, the patient dies.

Manifestations of chronic myelogenous leukemia

Chronic phase symptoms:

• Signs associated with dysfunction of platelets and white blood cells : various bleeding or, conversely, the formation of blood clots.
• Signs associated with an increase in the number of platelets and, as a result, an increase in blood clotting : circulatory disorders in the brain (headaches, dizziness, memory loss, attention, etc.), myocardial infarction, visual impairment, shortness of breath.

Symptoms of the acceleration phase

Symptoms of end-stage chronic myelogenous leukemia:

• Sharp weakness , a significant deterioration in general well-being.
• Prolonged aching pain in the joints and bones . Sometimes they can be very strong. This is due to the growth of malignant tissue in the red bone marrow.
• pouring sweats .
• Periodic unreasonable rise in temperature up to 38 - 39⁰C, during which there is a strong chill.
• Weight loss .
• Increased bleeding , the appearance of hemorrhages under the skin. These symptoms result from a decrease in the number of platelets and a decrease in blood clotting.
• Rapid enlargement of the spleen : the stomach increases in size, there is a feeling of heaviness, pain. This is due to the growth of tumor tissue in the spleen.

Diagnosis of the disease

Which doctor should I contact if I have symptoms of chronic myelogenous leukemia?

A hematologist is engaged in the treatment of blood diseases of a tumor nature. Many patients initially turn to a general practitioner, who then sends them for a consultation with a hematologist.

Examination at the doctor's office

• Questioning the patient . The doctor finds out the patient's complaints, specifies the time of their occurrence, asks other necessary questions.
• Feeling the lymph nodes : submandibular, cervical, axillary, supraclavicular and subclavian, ulnar, inguinal, popliteal.
• Feeling the abdomen to determine the enlargement of the liver and spleen. The liver is felt under the right rib in the supine position. The spleen is on the left side of the abdomen.

When can a doctor suspect chronic myelogenous leukemia in a patient?

• General blood analysis . An increased number of leukocytes and a large number of their immature forms are found in it.
• abdominal ultrasound . An increase in the size of the spleen is revealed.

How is a complete examination for suspected chronic myeloid leukemia performed??

Study Title	Description	What reveals?
General blood analysis	Routine clinical examination, performed when any disease is suspected. A general blood test helps to determine the total content of leukocytes, their individual varieties, immature forms. Blood for analysis is taken from a finger or a vein in the morning.	The result depends on the phase of the disease. chronic phase: a gradual increase in the content of leukocytes in the blood due to granulocytes; the appearance of immature forms of leukocytes; an increase in the number of platelets. Acceleration phase: the content of leukocytes in the blood continues to increase; the proportion of immature white blood cells increases to 10 - 19%; the content of platelets can be increased or decreased. Terminal phase: the number of immature leukocytes in the blood increases by more than 20%; decrease in the number of platelets;
Puncture and biopsy of red bone marrow	Red bone marrow is the main hematopoietic organ of a person, which is located in the bones. During the study, a small fragment is obtained using a special needle and sent to the laboratory for examination under a microscope. Carrying out the procedure: The puncture of the red bone marrow is carried out in a special room in compliance with the rules of asepsis and antisepsis. The doctor performs local anesthesia - punctures the puncture site with an anesthetic. A special needle with a limiter is inserted into the bone so that it penetrates to the desired depth. The puncture needle is hollow inside, like a syringe needle. It collects a small amount of red bone marrow tissue, which is sent to a laboratory for examination under a microscope. For puncture choose bones that are shallow under the skin: sternum; wings of the pelvic bones; calcaneus; tibial head; vertebrae (rare).	In the red bone marrow, approximately the same picture is found as in the general blood test: a sharp increase in the number of precursor cells that give rise to leukocytes.
Cytochemical study	When special dyes are added to blood and red bone marrow samples, certain substances may react with them. This is the basis of the cytochemical study. It helps to establish the activity of certain enzymes and serves to confirm the diagnosis of chronic myeloid leukemia, helps to distinguish it from other types of leukemia.	In chronic myeloid leukemia, a cytochemical study reveals a decrease in the activity of a special enzyme in granulocytes - alkaline phosphatase .
Blood chemistry	In chronic myeloid leukemia, the content of certain substances in the blood changes, which is an indirect diagnostic sign. Blood sampling for analysis is carried out from a vein on an empty stomach, usually in the morning.	Substances, the content of which in the blood is increased in chronic myeloid leukemia: vitamin B 12 ; lactate dehydrogenase enzymes; transcobalamin; uric acid.
Cytogenetic study	During a cytogenetic study, the entire genome (a set of chromosomes and genes) of a person is studied. For research, blood is used, which is taken from a vein into a test tube and sent to the laboratory. The result is usually ready in 20-30 days. The laboratory uses special modern tests, during which various parts of the DNA molecule are detected.	In chronic myeloid leukemia, a cytogenetic study reveals a chromosomal disorder, which was called Philadelphia chromosome . In the cells of patients, chromosome number 22 is shortened. The missing piece is attached to chromosome 9. In turn, a fragment of chromosome #9 is attached to chromosome #22. There is a kind of exchange, as a result of which the genes begin to work incorrectly. The result is myelogenous leukemia. Other pathological changes on the part of chromosome No. 22 are also detected. By their nature, one can partially judge the prognosis of the disease.
Ultrasound of the abdominal organs.	Ultrasonography is used in patients with myelogenous leukemia to detect enlargement of the liver and spleen. Ultrasound helps to distinguish leukemia from other diseases.

Laboratory indicators

• Leukocytes: significantly increased from 30.0 10 9 /l to 300.0-500.0 10 9 /l
• Shift of the leukocyte formula to the left: young forms of leukocytes predominate (promyelocytes, myelocytes, metamyelocytes, blast cells)
• Basophils: increased amount of 1% or more
• Eosinophils: increased level, more than 5%

• Platelets: normal or elevated

• Alkaline phosphatase of leukocytes is reduced or absent.

• A genetic blood test reveals an abnormal chromosome (Philadelphia chromosome).

Symptoms

• Long time without symptoms (3 months to 2 years)
• Heaviness in the left hypochondrium (due to an increase in the spleen, the higher the level of leukocytes, the larger its size).
• Weakness
• Decreased performance
• sweating
• weight loss

• spleen infarction - acute pain in the left hypochondrium, temperature 37.5 -38.5 ° C, sometimes nausea and vomiting, touching the spleen is painful.

• Priapism is a painful, excessively long erection.

• Decreased effectiveness of drugs (cytostatics)
• Anemia develops
• Increases the percentage of blast cells in the blood
• General condition worsens
• Enlarged spleen

• Symptoms correspond to the clinical picture in acute leukemia ( see Acute lymphocytic leukemia).

How is myeloid leukemia treated?

Treatment of the disease should be started immediately after the diagnosis is established. The prognosis largely depends on the quality and timeliness of therapy.

Treatment includes various methods: chemotherapy, radiation therapy, removal of the spleen, bone marrow transplant.

Drug treatment

• Classic drugs: Mielosan (Mileran, Busulfan), Hydroxyurea (Gidrea, Litalir), Cytosar, 6-mercaptopurni, alpha-interferon.
• New drugs: Glivec, Sprycell.

Bone marrow transplantation

Red bone marrow transplantation is the most effective treatment for chronic myeloid leukemia. More than half of transplanted patients experience sustained improvement over 5 years or longer.

Most often, recovery occurs when red bone marrow is transplanted to a patient younger than 50 years in the chronic phase of the disease.

Stages of red bone marrow transplantation:

• Finding and preparing a donor. The best donor of red bone marrow stem cells is a close relative of the patient: twin, brother, sister. If there are no close relatives, or they are not suitable, a donor is sought. A series of tests are carried out to make sure that the donor material will take root in the patient's body. Today, large donor banks have been established in developed countries, which contain tens of thousands of donor samples. This gives a chance to find suitable stem cells faster.
• Patient preparation. Usually this stage lasts from a week to 10 days. Radiation therapy and chemotherapy are carried out to destroy as many tumor cells as possible, to prevent rejection of donor cells.
• The actual red bone marrow transplant. The procedure is similar to a blood transfusion. A catheter is inserted into the patient's vein, through which stem cells are injected into the bloodstream. They circulate in the bloodstream for some time, and then settle in the bone marrow, take root there and begin to work. To prevent rejection of the donor material, the doctor prescribes anti-inflammatory and anti-allergic drugs.
• Decreased immunity. Donor cells of the red bone marrow cannot take root and begin to function immediately. This takes time, usually 2-4 weeks. During this period, the patient's immunity is greatly reduced. He is placed in a hospital, completely protected from contact with infections, antibiotics and antifungal agents are prescribed. This period is one of the most difficult. The body temperature rises sharply, chronic infections can be activated in the body.
• Engraftment of donor stem cells. The patient's condition begins to improve.
• Recovery. Within months or years, red bone marrow function continues to recover. Gradually, the patient recovers, his working capacity is restored. But he still needs to be under medical supervision. Sometimes the new immunity cannot cope with some infections, in this case, vaccinations are given about a year after the bone marrow transplant.

Radiation therapy

In what phase of the disease is radiation therapy used?

Radiation therapy is used in the advanced stage of chronic myeloid leukemia, which is characterized by signs:

• Significant proliferation of tumor tissue in the red bone marrow.
• The growth of tumor cells in tubular bones 2 .
• Great enlargement of the liver and spleen.

Gamma therapy is used - irradiation of the spleen area with gamma rays. The main task is to destroy or stop the growth of malignant tumor cells. The radiation dose and radiation regimen is determined by the attending physician.

Removal of the spleen (splenectomy)

The operation is usually performed in the terminal phase of the disease. Together with the spleen, a large number of tumor cells are removed from the body, thereby facilitating the course of the disease. After surgery, the effectiveness of drug therapy usually increases.

What are the main indications for surgery?

• Rupture of the spleen.
• The threat of rupture of the spleen.
• A significant increase in the size of the organ, which leads to severe discomfort.

Cleansing the blood of excess white blood cells (leukapheresis)

With the development of a blast crisis, the treatment will be the same as for acute leukemia (see acute lymphocytic leukemia).

Leukocytepheresis - a treatment procedure plasmapheresis (purification of the blood). A certain amount of blood is taken from the patient and passed through a centrifuge, in which it is cleaned of tumor cells.

In what phase of the disease is leukocytapheresis performed?
As well as radiation therapy, leukocytapheresis is performed during the advanced stage of myeloid leukemia. Often it is used in cases where there is no effect from the use of drugs. Sometimes leukocytapheresis complements drug therapy.

There are many diagnoses, the name of which says little to ordinary citizens. One such disease is chronic myelogenous leukemia. Reviews of patients with this disease, however, are able to attract attention, since this disease can not only cause significant damage to health, but also lead to a fatal outcome.

The essence of the disease

If one hears such a diagnosis as "chronic myeloid leukemia", then it is important to understand that we are talking about a serious tumor disease of the hematopoietic system, in which the hematopoietic stem cells of the bone marrow are affected. It can be attributed to the group of leukemias, which are characterized by large formations of granulocytes in the blood.

At the very beginning of its development, myeloid leukemia manifests itself through an increase in the number of leukocytes, reaching almost 20,000 / μl. At the same time, in the progressive phase, this figure changes to 400,000 / μl. It is worth noting the fact that both in the hemogram and in the myelogram, the predominance of cells with different degrees of maturity is recorded. These are promyelocytes, metamyelocytes, stab and myelocytes. In the case of myeloid leukemia, changes in the 21st and 22nd chromosomes are detected.

This disease in most cases leads to a noticeable increase in the content of basophils and eosinophils in the blood. This fact is evidence that one has to deal with a severe form of the disease. In patients who suffer from such an oncological disease, splenomegaly develops, and a large number of myeloblasts are recorded in the bone marrow and blood.

How does the onset of the disease occur?

Chronic myeloid leukemia pathogenesis is quite interesting. Initially, a somatic mutation of a pluripotent hematopoietic blood stem cell can be identified as a triggering factor in the development of this disease. The main role in the mutation process is played by the cross-translocation of chromosomal material between the 22nd and 9th chromosomes. In this case, the formation of the Ph-chromosome occurs.

There are cases (no more than 5%) when the Ph chromosome cannot be detected during a standard cytogenetic study. Although a molecular genetic study reveals an oncogene.

Chronic myeloid leukemia can also develop due to exposure to various chemicals and radiation. Most often this disease is diagnosed in adulthood, it is extremely rare in adolescents and children. With regard to gender, this type of tumor is recorded with the same frequency in both men and women aged 40 to 70 years.

Despite all the experience of doctors, the etiology of the development of myeloid leukemia is still not completely clear. Experts suggest that acute and chronic myeloid leukemia develops due to a violation of the chromosomal apparatus, which, in turn, is caused by the influence of mutagens or hereditary factors.

Speaking about the impact of chemical mutagens, it is worth paying attention to the fact that enough cases have been recorded when people who were exposed to benzene or used cytostatic drugs (Mustargen, Imuran, Sarcozoline, Leukeran, etc.) developed myeloid leukemia.

Chronic myeloid leukemia: stages

With such a diagnosis as "myeloid leukemia", there are three stages in the development of this disease:

Initial. It is characterized by an increase in the spleen and a stable injection of leukocytes in the blood. The patient's condition is considered in dynamics, without applying radical treatment measures. The disease, as a rule, is diagnosed already at the stage of total generalization of the tumor in the bone marrow. At the same time, in the spleen, and in some cases in the liver, there is an extensive proliferation of tumor cells, which is characteristic of the advanced stage.

Expanded. Clinical signs at this stage begin to dominate, and the patient is prescribed treatment using specific drugs. At this stage, the myeloid tissue in the bone marrow, liver, and spleen expands, and the fat in the flat bones is virtually completely replaced. There is also a sharp predominance of the granulocytic lineage and three-line proliferation. It should be noted that in the advanced stage, the lymph nodes are extremely rarely affected by the leukemic process. In some cases, myelofibrosis may develop in the bone marrow. There is a possibility of developing pneumosclerosis. As for the infiltration of the liver by tumor cells, in most cases it is quite pronounced.

Terminal. At this stage of the development of the disease, thrombocytopenia and anemia progress. Manifestations of various complications (infections, bleeding, etc.) become apparent. It is not uncommon for a second tumor to develop from immature stem cells.

What life expectancy should you expect?

If we talk about people who had to deal with chronic myeloid leukemia, it is worth noting that modern treatment methods have significantly increased the chances of such patients for a relatively long life. Due to the fact that discoveries were made in the field of pathogenetic mechanisms of the development of the disease, which made it possible to develop drugs that can act on the mutated gene, with such a diagnosis as chronic myeloid leukemia, the life expectancy of patients can be 30-40 years from the moment the disease is detected. But this is possible provided that the tumor was benign (slow enlargement of the lymph nodes).

In the case of the development of a progressive or classic form, the average is from 6 to 8 years from the moment the disease was diagnosed. But in each individual case, the number of years that the patient can enjoy is tangibly influenced by the measures that were taken in the course of treatment, as well as the form of the disease.

On average, according to statistics, up to 10% of patients die during the first two years after the detection of the disease, and 20% in subsequent years. Many patients with myeloid leukemia die within 4 years after the diagnosis was made.

Clinical picture

The development of a disease such as chronic myeloid leukemia is gradual. At first, the patient feels a deterioration in his general state of health, fatigue, weakness, and in some cases moderate pain in the left hypochondrium. After the study, an increase in the spleen is often recorded, and a significant neutrophilic leukocytosis is detected in a blood test, characterized by a shift in the leukocyte formula to the left due to the action of myelocytes with an increased content of basophils, eosinophils and platelets. When the time comes for a detailed picture of the disease, patients experience disability due to sleep disturbance, sweating, a steady increase in general weakness, a significant increase in temperature, pain in the spleen and bones. There is also a loss of weight and appetite. At this stage of the disease, the spleen and liver are greatly enlarged.

At the same time, chronic myeloid leukemia, the symptoms of which differ depending on the stage of development of the disease, already in the initial stage leads to the predominance of eosinophils, granular leukocytes and basophils in the bone marrow. Such growth occurs due to a decrease in other leukocytes, normoblasts and erythrocytes. If the process of the course of the disease begins to worsen, then the number of immature myeloblasts and granulocytes increases significantly, and hemocytoblasts begin to appear.

The blast crisis in chronic myeloid leukemia leads to total power metaplasia. In this case, there is a high fever, during which there are no signs of infection. Hemorrhagic syndrome develops (intestinal, uterine, mucous bleeding, etc.), leukemids in the skin, ossalgia, lymph nodes increase, complete resistance to cytostatic therapy and infectious complications are recorded.

If it was not possible to significantly affect the course of the disease (or such attempts were not made at all), then the condition of the patients will progressively worsen, and thrombocytopenia will appear (the phenomena of hemorrhagic diathesis make themselves felt) and severe anemia. Due to the fact that the size of the liver and spleen are growing rapidly, the volume of the abdomen increases markedly, the state of the diaphragm becomes high, the abdominal organs are compressed, and, as a result of these factors, the respiratory excursion of the lungs begins to decrease. Moreover, the position of the heart changes.

When chronic myeloid leukemia develops to this level, against the background of pronounced anemia, dizziness, shortness of breath, palpitations and headache appear.

Monocytic crisis in myelogenous leukemia

Regarding the topic of monocytic crisis, it should be noted that this is a rather rare phenomenon, during which young, atypical and mature monocytes appear and grow in the bone marrow and blood. Due to the fact that the bone marrow barriers are broken, fragments of megakaryocyte nuclei appear in the blood at the terminal stage of the disease. One of the most important elements of the terminal stage in a monocytic crisis is the inhibition of normal hematopoiesis (regardless of the morphological picture). The disease process is aggravated due to the development of thrombocytopenia, anemia and granulocytopenia.

Some patients may have a rapid enlargement of the spleen.

Diagnostics

The fact of the progression of such a disease as chronic myeloid leukemia, the prognosis of which can be quite gloomy, is determined by a whole range of clinical data and specific changes in the process of hematopoiesis. In this case, histological studies, histograms and myelograms are necessarily taken into account. If the clinical and hematological picture does not look clear enough and there is not enough data to make a confident diagnosis, then doctors focus on the detection of the Ph chromosome in monocytes, megakaryocytes, erythrocytes and granulocytes of the bone marrow.

In some cases, it is necessary to differentiate chronic myeloid leukemia. Diagnosis, which can be defined as differential, is focused on identifying a typical picture of the disease with hyperleukocytosis and splenomegaly. If the variant is atypical, then a histological examination of the punctate of the spleen is performed, as well as a study of the myelogram.

Certain difficulties can be observed when patients are admitted to the hospital in a state of blast crisis, the symptoms of which are very similar to myeloid leukemia. In such a situation, the data of a thoroughly collected anamnesis, cytochemical and cytogenetic studies significantly help. Often, chronic myeloid leukemia has to be differentiated from osteomyelofibrosis, in which one can observe intense myeloid metaplasia in the lymph nodes, spleen, liver, as well as significant splenomegaly.

There are situations, and they are not uncommon, when a blood test helps to identify chronic myeloid leukemia in patients who underwent a routine examination (in the absence of complaints and asymptomatic course of the disease).

Diffuse myelosclerosis can be ruled out by X-ray examination of the bones, which reveals multiple areas of sclerosis in flat bones. Another disease that, although rare, still has to be differentiated from myeloid leukemia, is hemorrhagic thrombocythemia. It can be characterized as leukocytosis with a shift to the left and an enlarged spleen.

Laboratory studies in the diagnosis of myeloid leukemia

In order to accurately determine the patient's condition if chronic myeloid leukemia is suspected, a blood test can be carried out in several directions:

Blood chemistry. It is used to detect abnormalities in the functioning of the liver and kidneys, which are the result of the use of certain cytostatic agents or were triggered by the spread of leukemic cells.

- Clinical blood test (complete). It is necessary to measure the level of various cells: platelets, leukocytes and erythrocytes. In most patients who have had to deal with a disease such as chronic myeloid leukemia, the analysis reveals a large number of immature white cells. Sometimes there may be a low platelet or red blood cell count. Such results are not the basis for determining leukemia without an additional test, which is aimed at examining the bone marrow.

Examination of bone marrow and blood samples under a microscope by a pathologist. In this case, the shape and size of the cells are studied. Immature cells are identified as blasts or myeloblasts. The number of hematopoietic cells in the bone marrow is also counted. The term "cellularity" applies to this process. In those with chronic myeloid leukemia, the bone marrow is usually hypercellular (a large accumulation of hematopoietic cells and a high content of malignant ones).

Treatment

With a disease such as chronic myeloid leukemia, treatment is determined depending on the stage of development of tumor cells. If we are talking about mild clinical and hematological manifestations in the chronic stage of the disease, then nutritious nutrition enriched with vitamins, regular dispensary observation and restorative therapy should be considered as topical therapeutic measures. Interferon can favorably influence the course of the disease.

In the case of the development of leukocytosis, doctors prescribe Mielosan (2-4 mg / day). If you have to deal with a higher leukocytosis, then the dose of Mielosan can rise to 6 and even 8 mg / day. It is worth waiting for the manifestation of a cytopenic effect no earlier than 10 days after the first dose of the drug. A decrease in the size of the spleen and a cytopenic effect occurs on average during the 3-6th week of treatment, if the total dose of the drug was from 200 to 300 mg. Further therapy involves taking 2-4 mg of Mielosan once a week, which at this stage has a supporting effect. If the first signs of exacerbation make themselves known, myelosanotherapy is performed.

It is possible to use a technique such as radiation therapy, but only if splenomegaly is determined as the main clinical symptom. For the treatment of patients whose disease is in a progressive stage, poly- and monochemotherapy is relevant. If significant leukocytosis is recorded, with insufficiently effective exposure to Mielosan, Myelobromol is prescribed (125-250 mg per day). At the same time, strict control of peripheral blood parameters is carried out.

In the case of the development of significant splenomegaly, "Dopan" is prescribed (once 6-10 g / day). Patients take the drug once for 4-10 days. The intervals between doses are determined depending on the degree and rate of decrease in the number of leukocytes, as well as the size of the spleen. As soon as the decrease in leukocytes reaches an acceptable level, the use of Dopan is stopped.

If the patient develops resistance to Dopan, Mielosan, radiation therapy and Myelobromol, Hexaphosphamide is prescribed for treatment. In order to effectively influence the course of the disease in the progressive stage, the TsVAMP and AVAMP programs are used.

If resistance to cytotoxic therapy develops in a disease such as chronic myeloid leukemia, treatment in the progression stage will focus on the use of leukocytophoresis in combination with a specific polychemotherapy regimen. As urgent indications for leukocytophoresis, clinical signs of stasis in the vessels of the brain (feeling of heaviness in the head, hearing loss, headaches) can be determined, which are caused by hyperthrombocytosis and hyperleukocytosis.

In the case of a blast crisis, various chemotherapy programs used for leukemia can be considered relevant. Indications for transfusion of erythrocyte mass, thromboconcentrate and antibiotic therapy are infectious complications, the development of anemia and thrombocytopenic hemorrhage.

Regarding the chronic stage of the disease, it is worth noting that at this stage of the development of myeloid leukemia, bone marrow transplantation is quite effective. This technique is able to ensure the development of clinical and hematological remission in 70% of cases.

An urgent indication for the use of splenectomy in chronic myeloid leukemia is the threat of rupture or the rupture of the spleen itself. Relative indications include severe abdominal discomfort.

Radiation therapy is indicated for those patients who have been diagnosed with life-threatening extramedullary tumor formations.

Chronic myeloid leukemia: reviews

According to patients, such a diagnosis is too serious to be ignored. By studying the testimonies of various patients, the real possibility of defeating the disease becomes apparent. For this, it is necessary to undergo a timely diagnosis and a course of subsequent treatment. Only with the participation of highly qualified specialists is there a chance to defeat chronic myeloid leukemia with minimal health losses.

Chronic myeloid leukemia is a blood disease of tumor etiology. With its development, uncontrolled growth and reproduction of all germ blood cells is observed. Pathological changes in one of the chromosomes cause the formation of a mutated gene, which causes a violation of hematopoiesis in the red bone marrow and, as a result, increased cell growth.

The International Classification of Diseases of the Tenth Revision (ICD 10) assigns the code C92 to the disease. It can occur in 3 forms, depending on the stage. Taking into account how timely chronic myeloid leukemia was diagnosed, the maximum life expectancy of the patient is determined.

Reasons for development

The growth and functioning of healthy cells in the body occurs on the basis of the information that the chromosomes contain. When a particular cell divides, it creates a new copy of the DNA in the chromosomes. If such a division process is disturbed, mutating genes can be formed, which affect the development of oncological pathologies.

In the human body there are genes that stimulate the process of cell development - oncogenes. It also contains genes that slow down their growth, which is necessary for cell death at the right time - suppressors. When the activity of such genes is disturbed, healthy cells degenerate into oncological ones and suppressors are turned off from this process.

Modern medicine does not have enough specific information about why chronic myeloid leukemia develops, including acute. This issue is under study. There are suggestions that some predisposing factors influence the development of the disease:

1. Effects on the body of radioactive irradiation. Proof of this can be called the case of Nagasaki and Hiroshima. The medical history (ICD 10 - C92) of the Japanese in the area of ​​the accident states that most of them were susceptible to the development of chronic myeloid leukemia.
2. Viral damage to the body, as well as electromagnetic rays and chemicals that affect the body. Such a factor as a potential cause of the development of the disease is still being considered by researchers today.
3. hereditary predisposition. People with congenital chromosomal abnormalities are at an increased risk of developing myeloid leukemia. In most cases, these are people who have been diagnosed with Down syndrome or Klinefelter syndrome.
4. Treatment of tumor-like neoplasms certain medicines by the type of cytostatics in combination with radiation.

All such predisposing factors cause a structural disorder of cellular chromosomes in the red bone marrow and the formation of new DNA with an abnormal structure. At the same time, the number of the latter begins to increase so much that they crowd out healthy cells. At this time, uncontrolled growth of abnormal cells, similar to cancer cells, is observed.

Stages of development of the disease

Most people (about 80%) go to the hospital already at the time when the disease becomes chronic. At this time, slightly pronounced symptoms of myeloid leukemia are observed, which are often confused with ordinary overwork: general malaise, decreased ability to work, increased sweating.

The chronic form of the disease can be asymptomatic for 2-3 months, and sometimes much longer - up to several years. In some cases, myelogenous leukemia is diagnosed quite by accident, by conducting a blood test to detect a different pathology in the body.

Chronic myeloid leukemia can be accompanied by complications in the form of an increase in the general temperature to high rates, pain in the left hypochondrium, etc. In the presence of complications, this form of the disease develops for 4 years or more.

If you do not start timely treatment of the disease of the chronic stage, it goes into stage 2 - acceleration. Immature leukocytes are intensively produced, reaching a volume of 10-19%. This stage lasts for about a year. At this stage of development, another symptomatology joins, which aggravates the general condition of the patient: anemia develops, the spleen enlarges, and the drugs used in the treatment do not bring the same effectiveness as at the initial stage of the development of the disease.

If treatment is not started at the acceleration stage, the disease passes into the terminal stage, the pathogenesis of which is characterized by an increase in the number of malignant cells in the bone marrow and the complete absence of healthy cells in it. In this case, the outcome is the least favorable and the treatment prescribed by the doctor often turns out to be ineffective.

Symptoms

Chronic myelocytic leukemia (CML) can have different symptoms, depending on the stage at which the disease develops. Symptoms common to all stages include:

• severe general malaise;
• weight loss;
• decrease or complete loss of appetite (depending on the stage of the disease);
• the spleen and liver in chronic myeloid leukemia increase;
• blanching of the skin;
• pain syndrome in the bones;
• increased sweating.

If we consider the clinic of the disease, taking into account its stage, it looks like this:

1. Chronic: rapid satiety during meals, pain in the left hypochondrium, shortness of breath and a feeling of lack of air during exercise, headache, impaired visual function. Men may experience prolonged painful erections.
2. Acceleration stage. At this stage, anemia of a progressive course develops, general pathological symptoms increase in intensity, pathological leukocyte cells are at an elevated level in the blood.
3. Terminal. The general condition of the patient worsens to critical indicators. There is a febrile syndrome, the general temperature rises to a maximum mark. Also, the development of terminal myelosis is characterized by bleeding through the mucous membranes, skin, intestines. Due to the increase in the spleen and hepatic lobes, pain occurs in the left hypochondrium and a feeling of heaviness.

Diagnostics

At different stages of the development of the disease, specific diagnostics are required. At the initial stage of the course, appoint:

1. Carrying out a general blood test. The study helps to identify a slight decrease in blood components: hemoglobin and red blood cells. Often their level remains normal at this stage of the disease. You can detect the presence of moderate thrombocytosis, basophilia, eosinophilia. The blood picture in chronic myeloid leukemia shows leukocytosis with indicators of 15-30 * 109 / l.
2. Conducting biochemical analysis. Diagnosis shows an increase in the amount of uric acid in the body.
3. Conducting a sternal puncture of the bone substance. Megakaryocytes are exceeded in their level of content, as well as granulocytic cells of young forms.

At the acceleration stage, it is necessary to carry out the following diagnostic measures:

At the terminal stage, pathology can be detected by:

1. Complete blood count, which helps to detect a critical decrease in the volume of red blood cells, platelets and hemoglobin, an increase in the volume of basophils up to 20%. Leukocytosis reaches 500-1000 * 109 / l.
2. Sternal puncture, which helps to identify a critical increase in the content of malignant cells in the medulla, as well as basophils and eosinophils.
3. Cytogenetic analysis, which helps to identify the presence of the Philadelphia chromosome in the body.

How to treat the disease

Myeloid blood disease requires specific treatment, the type of which is determined taking into account the stage of the course. In the event that the clinic of the disease is not very pronounced or is completely absent, they prescribe the observance of the correct diet, the intake of vitamin preparations, and general strengthening procedures. In this case, systematic monitoring by the attending physician is required.

If pronounced symptoms have joined, cytostatic drugs that block the growth of pathological cells are prescribed. Despite the high effectiveness of drugs, they can cause side effects: nausea, general malaise, hair loss, inflammation of the stomach or intestines.

In severe cases, bone marrow transplantation and blood transfusion are performed. Sometimes such treatment helps to permanently save a person from the disease. The only condition is the complete compatibility of the donor substance with the patient's bone marrow.

Folk remedies in the treatment of chronic leukemia will not be effective. These are used only to strengthen human immunity and increase the body's defenses. Gleevec is considered an excellent drug in the treatment of the disease, with which you can cause hematological remission of the pathology. The substances that make up the drug block and destroy the Philadelphia chromosome.

In an extremely severe case, a complete resection (removal) of the spleen is necessary, which improves the general condition of the patient and increases the effectiveness of the therapy.