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Myeloid leukemia symptoms. Chronic myeloid leukemia - life expectancy at different stages of the course of the disease. Causes of Chronic Myeloid Leukemia

Chronic myeloid leukemia is determined by the growth and division of blood cells, and this happens uncontrollably. Simply put, it is a malignant blood disease of a clonal nature, in which cancer cells are able to mature to mature forms. A synonym for chronic myeloid leukemia is chronic myeloid leukemia, popularly called "leukemia".

The bone marrow produces blood cells, with myeloid leukemia, immature cells are formed in the blood, which doctors call blasts, so in some cases this disease is called chronic myeloid leukemia. Blasts gradually replace healthy blood cells, and with the blood flow penetrate into all organs of the human body.

A human cell contains 46 chromosomes. Each of them has sections that are located in a certain sequence - they are called genes. Each segment (gene) is responsible for the production of proteins (only one type) that the body needs for life.

Under the influence of provoking factors - radiation and other factors, including unknown ones, two chromosomes exchange their sections with each other. The result is an altered chromosome, which scientists have named the Philadelphia chromosome (because it was first discovered there). It is known that this chromosome regulates the production of a certain protein that causes mutation processes in the cell, that is, it allows it to divide uncontrollably.

Atypical cells often appear in a healthy body, but the immune system quickly destroys them. But the Philadelphia chromosome gene gives it stability, and the body's defenses cannot destroy it. As a result, after some time, the number of altered cells exceeds the number of healthy and unchanged ones, so chronic myeloid leukemia develops.

Reasons for the development of the disease

Until the end, the etiology of CML has not yet been studied, scientists around the world are struggling with this issue, as soon as the cause of the disease is revealed, a cure will appear from this disease. All cells originate from stem cells, which are mainly localized in the bone marrow, after their maturation is completed, the cells begin their functions.

Leukocytes - protect against infection, erythrocytes deliver oxygen and other substances to all cells, platelets - do not allow bleeding, forming clots. As a rule, it is the leukocyte cell that begins to divide uncontrollably, however, not all cells mature, therefore a large number of mature and immature cells appear in the bloodstream.

Currently, only indirect reasons for which the disease develops are known:

  • Stem cells change their structure, this mutation gradually progresses, and as a result, blood cells become pathological. They are called "pathological clones". Cytostatic drugs cannot eliminate them or stop them from dividing.
  • Harmful chemicals.
  • Radiation. Chronic myeloid leukemia is sometimes diagnosed in patients who have received radiation therapy to treat other malignant tumors.
  • Prolonged exposure to the body of cytotoxic drugs, which are also used to treat cancer. There is a whole list of drugs that can provoke chronic myeloid leukemia.
  • Down Syndrome.
  • Pathological effects of aromatic carbohydrates.
  • Viruses.

However, all these reasons cannot give a complete picture of the etiology of the disease, since they are only indirect, the true cause of science is not yet known.

Types of leukemia

Myeloid leukemia is distinguished by the nature of the course and by the type of pathological cells. According to the course of the disease, acute and chronic forms are distinguished. Chronic myeloid leukemia is characterized by a slower development of pathology, and also certain changes occur in the blood, which does not happen in an acute form.

According to the cellular composition, leukemia is divided into:

  • promyelocytic;
  • myelomonocytic, which in turn is also divided into several subspecies;
  • myelomonoblastic;
  • basophilic;
  • megakaryoblastic;
  • erythroid leukemia.

As for the chronic form, it is subdivided into juvenile, myelocintra, myelomonocintar (CMML), neutrophilic and primary.

Chronic myelomonocytic leukemia differs from chronic myeloid leukemia in that the largest leukocytes (monocytes) that do not have granules are cloned and enter the bloodstream still immature.

Stages of chronic myeloid leukemia

Chronic myeloid leukemia occurs in three stages:

  • initial;
  • deployed;
  • terminal.

If at the initial stage of the disease the patient does not receive adequate treatment, then myeloid leukemia gradually passes through all three stages, however, with timely and proper treatment, the disease can be slowed down at the initial or advanced stage.

The chronic (initial) stage can be observed for a long time, while the symptoms are practically absent and the presence of pathology can only be determined by passing a blood test. The patient may feel only some malaise, which, as a rule, is not paid attention to. Sometimes, after eating, there may be a feeling of oversaturation, which occurs due to an enlarged spleen.

The acceleration phase (detailed stage) is the next stage of the disease. With its onset, the clinical signs become more pronounced, which indicates that the leukemic process is developing. The patient develops profuse sweating, loss of strength, fever rises, weight decreases, pains appear in the left side under the ribs. In addition, heart pain, arrhythmia may appear - this indicates that the process has switched to the cardiovascular system.

The final stage of the disease is terminal (blast crisis). The patient's condition deteriorates sharply, the temperature continues to rise and no longer drops to normal levels. At this stage, the patient's body no longer responds to drug treatment, infections often join the process, they, as a rule, become the causes of death.

Clinical picture

The chronic form of myeloid leukemia is diagnosed in 15% of all cases of the disease. When diagnosed with chronic myeloid leukemia, the symptoms are initially not pronounced, the disease can continue asymptomatically for about 4-5 years, in some cases up to 10 years. The first striking symptom that a person can pay attention to is an increase in temperature for no apparent reason. An increase in temperature occurs due to an increase in the spleen and liver, which can cause some pain and discomfort in the right and left sides.

On palpation, the organs are painful. If the basophils in the blood increase significantly, the patient may develop itching and a feeling of heat, and if the terminal stage is close, joint pain may occur. In some cases, there is a risk of spleen infarction, if there is damage to the brain centers, then paralysis is possible. Lymph nodes are enlarged.

One form of chronic myeloid leukemia is juvenile myelosis. It is diagnosed in preschool children. This disease does not have an acute form, and the symptoms increase gradually:

  • the child is not active;
  • often suffers from infectious diseases;
  • has a poor appetite and is not gaining weight well;
  • development is slowed down;
  • nosebleeds are often observed.

Diagnosis of the disease

Most often, a blood test helps to suspect the disease, in addition, the doctor may be alerted by hepatomegaly and an enlarged spleen. The hematologist may refer the patient for ultrasound and genetic testing.

The patient's blood is subjected to the following diagnostics:

  • general analysis;
  • biochemical;
  • cytogenetic;
  • cytochemical.

A detailed blood test helps to trace the dynamics of the cellular composition. If the patient has the initial stage of the disease, then healthy, mature blood cells are evaluated and the number of immature blood structures is determined. In the accelerated stage, the analysis shows an increase in immature blood cells and a sharp change in the level of platelets. When blasts reach 20%, it can be said that the final stage of the disease has come.

Biochemical analysis determines the level of uric acid and other indicators that are characteristic of chronic myeloid leukemia. Cytochemistry is performed to differentiate the chronic form of leukemia from other forms of the disease.

During a cytogenetic study, specialists detect atypical chromosomes in blood cells. This is necessary not only for a melting diagnosis, but also for predicting the course of the disease.

Biopsy - needed to identify atypical cells, a specialist takes material from the femur for analysis. Ultrasound, CT and MRI give an idea of ​​the size of the liver and spleen, which also helps to distinguish the chronic form of sapwood from other forms of leukemia.

Treatment of the disease

When diagnosed with chronic myeloid leukemia, treatment is determined according to the stage of the disease. If hematological and symptomatic manifestations are not pronounced, then experts recommend good nutrition, vitamin therapy, restorative measures, as well as regular examinations. In other words, the tactics of monitoring the disease and strengthening the immune forces of the body are chosen.

Some doctors talk about the positive dynamics of the course of the disease when using interferon. If the patient is worried about nose (or other) bleeding or he has become more likely to get sick with infectious diseases, then strengthening measures alone will not be enough, more aggressive treatment must be taken.

At later stages of the disease, cytostatics are used, which block the growth of all cells. These are essentially cellular poisons, of course they inhibit the growth of cancer cells, but also cause severe adverse reactions in the body. This is nausea, and feeling unwell, and hair loss, and inflammatory processes in the intestines and stomach. Bone marrow transplantation and blood transfusion are practiced. In some cases, bone marrow transplantation can completely cure the patient, however, for the success of this operation, a bone marrow donor that is absolutely compatible with the patient is required.

It is impossible to treat myeloid leukemia on its own or with the help of traditional medicine. Medicinal herbs only help to strengthen the patient's body and increase his immunity. In the final stage of the disease, drugs are prescribed that are used in acute leukemia.

Studies conducted at the end of the last century proved that Imatinib (Gleevec) can lead to hematological remission. This is due to the fact that the Philadelphia chromosome disappears in the blood, which is the cause of the development of chronic myeloid leukemia. To date, relatively little experience has been accumulated in order to be able to discuss the advantages and disadvantages of this drug. But it is safe to say that it is superior to previously known drugs that have been used to treat chronic myeloid leukemia.

In extreme cases, the patient's spleen is removed, as a rule, such an intervention is carried out with a blast crisis. After removal of the hematopoietic organ, the course of the disease improves, and the effectiveness of drug treatment also increases.

Provided that the level of leukocytes increases extremely high, patients undergo leukopheresis. In fact, this procedure is similar to plasma cleansing. Often this procedure is prescribed in combination with drug treatment.

Forecasts for life

The prognosis for the chronic course of the disease is unfavorable, since this disease is associated with life-threatening ailments. The lethal outcome most often occurs in the accelerated and final stages of the disease. The average life expectancy for patients is 2 years.

After a blast crisis, patients die in about six months, but if remission is achieved, then life expectancy increases by about a year. However, you should not give up, no matter at what stage the disease proceeds, there is always a chance to prolong life. Perhaps the statistics do not include isolated cases when remission lasted for years, besides, scientists do not stop conducting research, and perhaps very soon a new method of effective treatment of chronic myeloid leukemia will appear.

Chronic myeloid leukemia (chronic myeloid leukemia) is a form of leukemia (leukemia) characterized by unregulated and accelerated proliferation (reproduction by division) of myeloid cells in the bone marrow with their subsequent accumulation in the blood. Chronic myeloid leukemia (CML) is more common in adults than in children.

Causes

The occurrence of CML is associated with a genetic anomaly, which is represented by a chromosomal translocation, manifested by the presence of the Ph "chromosome (Philadelphia chromosome) in the karyotype.

Symptoms of Chronic Myeloid Leukemia

In the chronic phase of CML, symptoms may be completely absent or mild. Perhaps a violation of the general condition - the appearance of malaise and weakness, decreased appetite, gradual weight loss, increased sweating at night. With an increase in the size of the spleen (splenomegaly), the patient may notice the appearance of heaviness or pain in the left side of the abdomen.

In the acceleration phase, there is an increase in the severity of the symptoms of the chronic stage. Sometimes it is in this phase that the first obvious signs of the disease appear, forcing a person to see a doctor for the first time.

Symptoms of the terminal stage of CML include a significant deterioration in general well-being, severe weakness, increased bleeding, the occurrence of hemorrhages on the body, rapid weight loss, the appearance of heavy sweats, prolonged pain in the joints and bones of a aching nature (in some cases, these pains become very strong). It is also possible to periodically causeless increase in body temperature up to 38 - 39⁰C with severe chills. Characteristic is the rapid increase in the size of the spleen.

Diagnostics

Diagnostic tests for CML include:


Classification

There are 3 variants of the course of chronic myeloid leukemia.

  • chronic phase - is a stage of relative stability. The patient at this stage may be disturbed by minimal symptoms.
  • acceleration phase - characterized by the activation of the pathological process. In the acceleration phase, the number of immature forms of leukocytes in the blood begins to increase rapidly. This stage can be called transitional from chronic to terminal.
  • terminal phase (blast crisis) is the final stage of chronic myeloid leukemia. This phase proceeds like acute leukemia and is characterized by rapid progression and low survival.

Patient's actions

Early stages of chronic myeloid leukemia are usually hidden. However, there are still some non-specific signs that make it possible to suspect CML.

The somatic features of CML include:


Of course, the presence of one or even several of the above signs in a person does not at all indicate that he is sick with chronic myeloid leukemia. . These symptoms are only possible manifestations of this disease. Moreover, these signs are non-specific - that is, they can appear with a huge number of other diseases, both serious and minor. However, if a person has most of these symptoms, this may be a reason to seek the advice of a doctor (hematologist).

Treatment of chronic myeloid leukemia

Therapeutic measures for chronic myeloid leukemia are aimed at reducing the growth of tumor cells and reducing the size of the spleen. The main treatments for CML include chemotherapy, splenectomy (removal of the spleen), radiation therapy, bone marrow transplant.

Complications

Characteristic complications of CML are hemorrhagic syndrome, infection, and damage to the respiratory system. The development of infectious and inflammatory processes (for example, pneumonia, bronchitis) is most often noted. As a rule, infectious complications of bacterial etiology occur, but fungal and viral infections are possible.

Prevention of chronic myeloid leukemia

Effective prevention of chronic myeloid leukemia has not been developed, because the exact causes of this pathology have not been identified.

Until recently, it was generally accepted that chronic myeloid leukemia is a disease that occurs more often in older men. Now doctors have come to the conclusion that both women and men have an equal chance of becoming victims of this disease. Why does this disease occur, who is at risk, and can it be cured?

The essence of the disease

In the human body, the bone marrow is responsible for the processes of hematopoiesis. Blood cells are produced there - erythrocytes, platelets and leukocytes. Most of all in the hemolymph of leukocytes. They are responsible for immunity. Chronic myeloid leukemia leads to the failure of these processes.

In a person suffering from this type of leukemia, the bone marrow produces leukocytes with pathology - oncologists call them blasts. They begin to multiply uncontrollably and leave the bone marrow without having time to mature. In fact, these are “immature” leukocytes that cannot perform protective functions.

Gradually, they are carried through the vessels to all human organs. The content of normal white blood cells in the plasma gradually decreases. The blasts themselves do not die - the liver and spleen cannot destroy them. The human immune system, due to the lack of leukocytes, ceases to fight allergens, viruses and other negative factors.

Causes of the disease

In the absolute majority of cases, chronic myeloid leukemia is caused by a gene mutation - a chromosomal translocation, which is commonly called the "Philadelphia chromosome".

Technically, the process can be described as follows: chromosome 22 loses one of the fragments that fuses with chromosome 9. A fragment of chromosome 9 attaches to chromosome 22. This is how the genes fail, and then the immune system.

Experts say that the occurrence of this type of leukemia is also affected by:

  • exposure to radiation. After the nuclear attacks on Hiroshima and Nagasaki, the incidence of CML among residents of Japanese cities increased significantly;
  • exposure to certain chemicals - alkenes, alcohols, aldehydes. Smoking affects the condition of patients negatively;
  • taking certain medications - cytostatics, if cancer patients take them along with undergoing radiation therapy;
  • radiotherapy;
  • hereditary genetic diseases - Klinefelter's syndrome, Down's syndrome;
  • viral diseases.

Important! CML mainly affects people older than 30-40 years, and the risk of getting sick increases with age, up to 80 years. It is rarely diagnosed in children.

There are, on average, one to one and a half cases of this disease per 100 thousand inhabitants of the Earth. In children, this figure is 0.1-0.5 cases per 100 thousand people.

How is the disease progressing?

Doctors distinguish three stages in the development of chronic myelogenous leukemia:

  • chronic stage;
  • accelerating stage;
  • terminal stage.

The first phase usually lasts two to three years and is most often asymptomatic. The manifestation of this disease is atypical and may not differ from general malaise. The disease is diagnosed by chance, for example, when a person comes to take a general blood test.

The first signs of the disease are general malaise, a feeling of fullness in the abdomen, heaviness in the left hypochondrium, decreased ability to work, low hemoglobin. On palpation, the doctor will find an enlarged spleen due to a tumor, and a blood test will reveal an excess of granulocytes and platelets. Men often experience long, painful erections.

The spleen enlarges, a person experiences problems with appetite, quickly becomes satiated, feels pains radiating to the back in the left side of the abdominal cavity.

Sometimes in the initial phase, the work of platelets is disrupted - their level rises, blood clotting increases. A person develops thrombosis, which is associated with headaches and dizziness. Sometimes the patient has shortness of breath with the most minimal physical exertion.

The second, accelerated stage occurs when the general condition of a person worsens, the symptoms become more pronounced, and laboratory tests record a change in the composition of the blood.

A person loses weight, becomes weak, dizzy and bleeding, and the temperature rises.

The body produces more and more myelocytes and white blood cells, and blasts appear in the bones. The body reacts to this by releasing histamine, so the patient begins to feel fever and itching. He begins to sweat profusely, especially at night.

The duration of the accelerative phase is from one to one and a half years. Sometimes a person begins to feel unwell only in the second stage and goes to the doctor when the disease is already progressing.

The third, terminal phase occurs when the disease passes into an acute stage.

A blast crisis occurs in chronic myeloid leukemia, when cells with pathology, almost completely, replace healthy ones in the organ responsible for hematopoiesis.

The acute form of chronic myeloid leukemia has the following symptoms:

  • severe weakness;
  • temperature rise to 39-40 degrees;
  • a person begins to lose weight rapidly;
  • the patient feels joint pain;
  • hypohidrosis;
  • hemorrhage and bleeding.

Acute myeloid leukemia often leads to splenic infarction - the tumor increases the risk of rupture.

The number of myeloblasts and lymphoblasts is growing. Blasts can turn into a malignant tumor - myeloid sarcoma.

Chronic myeloid leukemia in the third stage is incurable, and only palliative therapy will prolong the patient's life for several months.

How to diagnose a disease?

Since at first the disease has non-specific signs, it is often discovered almost by accident when a person comes, for example, to take a complete blood count.

A hematologist with suspicion of oncology should not only conduct a survey and examine his lymph nodes, but also palpate the abdomen to understand if the spleen is enlarged and if there is a tumor in it. To confirm or refute the suspicions, the subject is sent for an ultrasound scan of the spleen and liver, as well as for a genetic study.

Methods for diagnosing chronic myeloid leukemia:

  • common and ;
  • bone marrow biopsy;
  • cytogenetic and cytochemical study;
  • Ultrasound of the abdominal organs, MRI, CT.

A general detailed blood test allows you to trace the dynamics of the development of all its components.

At the first stage, it will allow you to determine the level of "normal" and "immature" white blood cells, granulocytes and platelets.

The accelerating phase is characterized by an increase in the level of leukocytes, an increase in the proportion of "immature" leukocytes up to 19 percent, as well as a change in the level of platelets.

If the proportion of blasts exceeds 20 percent, and the number of platelets decreases, then the third stage of the disease has begun.

Biochemical analysis will help to establish the presence in the blood of substances that are characteristic of this disease. We are talking about uric acid, vitamin B12, transcobalamin and others. Biochemistry determines whether there are malfunctions in the work of the lymphoid organs.

If a person has chronic myeloid leukemia in the blood, the following occurs:

  • significant increase;
  • the predominance of "immature" forms of leukocytes - blast cells, myelocytes, pro- and metamyelocytes.
  • increased content of baso- and eosinophils.

A biopsy is needed to determine the presence of abnormal cells. The doctor uses a special needle to take the brain tissue (a suitable place for puncture is the femur).

Cytochemical examination distinguishes chronic myeloid leukemia from other types of leukemia. Doctors add reagents to the blood and tissue obtained from a biopsy and see how the blood bodies behave.

Ultrasound and MRI give an idea of ​​the size of the abdominal organs. These studies help differentiate the disease from other types of leukemia.

Cytogenetic research helps to find abnormal chromosomes in blood cells. This method allows not only to reliably diagnose the disease, but also to predict its development. To detect an abnormal, or "Philadelphia" chromosome, the hybridization method is used.

Treatment of the disease

Treatment for chronic myeloid leukemia has two main goals: to shrink the spleen and to stop the bone marrow from making abnormal cells.

Oncologists-hematologists use four main methods of treatment:

  1. Radiation therapy;
  2. bone marrow transplant;
  3. Splenectomy (removal of the spleen)
  4. Leukapheresis.

Depends on the individual characteristics of the patient's body, as well as on the neglect of the disease and symptoms.

In the early stages in the treatment of leukemia, doctors prescribe drugs to strengthen the body, vitamins and a balanced diet to their wards. A person must also adhere to the regime of work and rest.

In the first stages, if the level of leukocytes rises, doctors often prescribe busulfan to the wards. If this gives results, the patient is transferred to maintenance therapy.

In the late phases, doctors use traditional drugs: Cytosar, Myelosan, Dazanitib, or modern drugs like Glivec and Sprycel. These drugs act on the oncogene. Together with them, patients are prescribed interferon. It should strengthen the human immune system.

Carefully! The doctor prescribes the regimen and dosage of medications. The patient is not allowed to do this on his own.

Chemotherapy usually comes with side effects. Taking medication often leads to indigestion, causes allergic reactions and convulsions, reduces blood clotting, provokes neuroses and depression, and leads to hair loss.

If the disease is in an advanced phase, hematologists prescribe several drugs at the same time. The duration of the course of intensive chemotherapy depends on how soon the laboratory parameters return to normal. Usually, a cancer patient should undergo three to four courses of chemotherapy per year.

If cytostatics and chemotherapy do not give results, and the disease continues to progress, the hematologist sends his ward to radiation therapy.

The indications for it are:

  • an increase in a tumor in the bone marrow;
  • enlargement of the spleen and liver;
  • if the blasts hit the tubular bones.

The oncologist must determine the mode and dose of radiation. The rays affect the tumor in the spleen. This stops the growth of oncogenes, or completely destroys them. Radiation therapy also helps relieve joint pain.

Irradiation is applied at the accelerating stage of the disease.

Bone marrow transplantation is one of the most effective treatments. It guarantees long-term remission in 70 percent of patients.

Bone marrow transplantation is a rather expensive method of treatment. It consists of several stages:

  1. Donor selection. The ideal option is when a close relative of a cancer patient becomes a donor. If he does not have brothers and sisters, then he has to be looked for in special databases. It is quite difficult to do this, since the chances that foreign elements will take root in the patient's body are less than if a member of his family became the donor. Sometimes it is the patient himself. Doctors can transplant peripheral cells into his bone marrow. The only risk is associated with a high probability that blasts will get there along with healthy leukocytes.
  2. Patient preparation. Before the operation, the patient must undergo a course of chemotherapy and radiation. This will kill a significant portion of the pathological cells and increase the chances that the donor cells will take root in the body.
  3. Transplantation. Donor cells are injected into a vein using a special catheter. First, they move through the vascular system, then they begin to act in the bone marrow. After transplantation, the doctor prescribes antiviral and anti-inflammatory drugs so that the donor material is not rejected.
  4. Working with the immune system. It is not immediately possible to understand whether donor cells have taken root in the body. After transplantation, two to four weeks should pass. Since the person's immunity is at zero, he is ordered to be in the hospital. He receives antibiotics, he is protected from contact with infectious agents. At this stage, the patient's body temperature rises, chronic diseases may worsen.
  5. post-transplantation period. When it is clear that foreign leukocytes have been accepted by the bone marrow, the patient's condition improves. Full recovery takes months or even years. All this time, a person must be observed by an oncologist and vaccinated, since his immune system will not be able to cope with many diseases. A special vaccine has been developed for people with weakened immune systems.

Transplantation is usually carried out at the first stage.

Removal of the spleen, or splenectomy, is used in the terminal stage if:

  • a spleen infarction has occurred, or there is a threat of its rupture;
  • if the organ has grown so much that it interferes with the functioning of neighboring abdominal organs.

What is leukapheresis? Leukocytopheresis is a procedure aimed at cleansing of pathological leukocytes. A certain amount of the patient's blood is driven through a special machine, where cancer cells are removed from it.

This treatment usually complements chemotherapy. Leukapheresis is performed when the disease progresses.

Survival Predictions

The healing of a cancer patient and his life expectancy depend on several factors.

The likelihood of recovery depends on what stage of chronic myelogenous leukemia was diagnosed. The sooner this is done, the better.

The chances of healing are reduced if the abdominal organs are seriously enlarged and protrude from under the edges of the costal arch.

A negative sign is leukocytosis, thrombocytopenia, as well as an increase in the content of blast cells.

The more manifestations and the patient has, the less favorable the prognosis will be.

With timely intervention, remission occurs in 70 percent of cases. After healing, the chances are high that the patient will live for several more decades.

The lethal outcome most often occurs in the accelerative and terminal stages, about seven percent of patients with chronic myeloid leukemia die in the first year after they were diagnosed with CML. The causes of death are severe bleeding and infectious complications due to weakened immunity.

Palliative therapy at the last stage after a blast crisis prolongs the life of the patient, at most, by half a year. The life expectancy of a cancer patient is calculated in a year if remission occurs after a blast crisis.

The content of the article

Chronic myeloid leukemia- a tumor, the cellular substrate of which is granulocytes, mainly neutrophils. Chronic myeloid leukemia develops in people of any age, more often at the age of 20-50 years, men and women get sick with the same frequency.

Etiology and pathogenesis of chronic myeloid leukemia

The influence of ionizing radiation and chemical agents on the development of chronic myeloid leukemia was noted. The disease is associated with a characteristic chromosomal abnormality - the Philadelphia (Ph ") chromosome, which appears as a result of reciprocal translocation of part of the long arm of chromosome 22 to chromosome 9. The biological mechanism of this chromosomal disorder is not well understood; according to modern data, chromosome rearrangements, including the occurrence of Ph " -chromosomes may be the result of activation of cellular oncogenes - genetic loci on human DNA, homologous to the DNA of viruses that cause malignant tumors in infected animals. The Ph"-chromosome is found in chronic myeloid leukemia in all cells of bone marrow lines, except for macrophages and T-lymphocytes, which indicates the likelihood of mutation of an early pluripotent precursor cell of hematopoiesis.
The development of chronic myeloid leukemia passes through two phases - chronic and acute (blast crisis). The power phase is the result of tumor progression, during this period the disease resembles acute leukemia, since blast cells are found in large numbers in the bone marrow and on the periphery. The malignant nature of the blast phase is reflected in cytogenetic changes: in addition to the Ph "-chromosome, aneuploidy and other karyotype disorders (trisomy of chromosomes 8, 17, 22) are often found.

Clinic of chronic myeloid leukemia

By the time of diagnosis, patients usually already have neutrophilic leukocytosis and an enlarged spleen. In the initial period, there may be no complaints and the disease is diagnosed by chance during a blood test, then general symptoms appear - weakness, fatigue, weight loss, discomfort in the abdominal cavity. Splenomegaly is often significant, and splenic infarcts occur. The liver is also usually enlarged, leukemic infiltration of other organs is possible - the heart, lungs, nerve roots.

Laboratory findings in chronic myelogenous leukemia

In the advanced stage of chronic myeloid leukemia, the number of leukocytes reaches 200-400-109/l, and in some cases - 800-1000-109/l. In the leukogram, a shift to myelocytes and promyelocytes is determined, single myeloblasts can occur, usually only with high leukocytosis.
An important hematological sign that appears already in the early stages of the disease is an increase in the content of basophils, as well as eosinophils of varying degrees of maturity. The number of platelets is normal or more often elevated over a long period of the disease; thrombocytopenia occurs in the final stage or as a result of chemotherapy treatment. Anemia also in most cases appears with the progression of the process. The development of anemia is possibly associated with the influence of a hyperplastic spleen, as well as latent hemolysis. In chronic myelogenous leukemia, leukocytosis may be accompanied by an increase in serum cyanocobalamin levels, as well as an increase in serum cyanocobalamin-binding capacity, hyperuricemia. Almost all patients have a significant decrease in the activity of alkaline phosphatase in granulocytes.
When examining the bone marrow obtained by sternal puncture, an increased number of cells (myelokaryocytes) is detected, while the cytological picture is almost identical to the picture of blood, but unlike smears from peripheral blood, there are erythroblasts and megakaryocytes. Chronic myeloid leukemia is characterized by an increase in the number of megakaryocytes, which persists for a significant period of the disease. A decrease in their number in the bone marrow occurs in parallel with a decrease in the number of blood platelets in the peripheral blood during an exacerbation of the leukemia process. In the bone marrow trepanate, even with a relatively low level of leukocytes in the blood, pronounced three-line hyperplasia of the myeloid tissue and the absence of fat are usually noted. stages of the disease find the predominance of myeloid cells.
Chronic myeloid leukemia is the only leukemia in which the chromosomal marker of leukemic cells (Ph "-chromosome) is detected with great constancy (in 90% of cases). Ph"-negative variant of chronic myeloid leukemia occurs in children and adults, is characterized by an unfavorable course and a short average life expectancy of patients The chronic phase of the disease lasts 3-5 years, after which an exacerbation of the disease occurs, a blast crisis develops, during which more than 85% of patients die. In some patients, the transition to the blast phase takes only a few weeks from the appearance of the first signs of the disease. Sometimes the disease is first diagnosed in this phase, the difference from acute leukemia is in the presence of the Ph "-chromosome. There is no specific test that can predict the onset of a blast crisis, at the same time, its early signs are known - increasing leukocytosis, splenomegaly, progressive anemia, thrombocytopenia refractory to previously effective therapy Some patients may develop extramedullary tumors, often in the lymph nodes or skin, or develop osteolysis.
The power phase in nature (origin) is myeloid or lymphoid. Myeloid crisis resembles acute myeloid leukemia, in 1/3 of cases blast cells have features of lymphoblasts, contain TdT and the common acute lymphocytic leukemia antigen; features of blast cells are important when choosing a therapy for blast crisis.

Diagnosis and differential diagnosis of chronic myeloid leukemia

Chronic myeloid leukocytosis is diagnosed based on the detection of pronounced neutrophilic leukocytosis with a shift to the left, splenomegaly. Differential diagnosis is carried out with myeloid-type leukemoid reactions associated with infections and tumors. In contrast to chronic myeloid leukemia, in leukemoid reactions, the activity of alkaline phosphatase in neutrophils is significantly increased and there is no Ph "-chromosome. A benign variant of the disease with a still small increase in the number of leukocytes and blood platelets has to be differentiated from similar myeloproliferative diseases - subleukemic myelosis and sometimes erythremia.

Tumor pathologies often affect the circulatory system. One of the most dangerous pathological conditions is chronic myeloid leukemia - this is characterized by erratic reproduction and growth of blood cells. This pathology is also called chronic myeloid leukemia.

The disease rarely affects representatives of childhood and adolescence, more often found in 30-70-year-old patients more often than males.

What is Chronic Myeloid Leukemia?

Basically, myeloid leukemia is a tumor that develops from early myeloid cells. Pathology is clonal in nature and among all hemoblastoses is about 8.9% of cases.

Myeloid chronic leukemia is characterized by asymptomatic development. For diagnosis, it is necessary to analyze a blood smear, as well as a bone marrow sample taken by aspiration (using a thin needle).

Chronic myelogenous leukemia is characterized by an increase in the blood composition of a specific type of white blood cells called granulocytes. They are formed in the red bone marrow and in large quantities penetrate into the blood in an unripe form. At the same time, the number of normal leukocyte cells decreases.

Causes

The etiological factors of chronic myeloid leukemia are still the subject of study and raise many questions among scientists.

It has been reliably revealed that the following factors affect the development of chronic myeloid leukemia:

  1. Radiation exposure. One of the proofs of such a theory is the fact that among the Japanese in the zone of destruction of the atomic bomb (the case with Nagasaki and Hiroshima), cases of the development of a chronic form of myeloid leukemia have become more frequent;
  2. Influence of viruses, electromagnetic rays and substances of chemical origin. Such a theory is controversial and has not yet received final recognition;
  3. hereditary factor. Studies have shown that individuals with chromosomal disorders are more likely to develop myeloid leukemia. Usually these are patients with Down's or Klinefelter's syndrome, etc.;
  4. Taking certain medications, such as cytostatics, used in the treatment of tumors in conjunction with radiation. In addition, alkenes, alcohols and aldehydes can be hazardous to health in this regard. A very negative effect on the well-being of patients with myeloid leukemia is nicotine addiction, which aggravates the condition of patients.

Structural disorders in the red bone marrow cell chromosomes lead to the birth of new DNA with an abnormal structure. As a result, clones of abnormal cells begin to be produced, which gradually replace normal cells to such an extent that their percentage in the red bone marrow becomes predominant.

As a result, abnormal cells multiply uncontrollably, similar to cancer cells. Moreover, their natural death according to generally accepted traditional mechanisms does not occur.

The concept of chronic myeloid leukemia and its causes, will tell the following video:

Once in the general circulation, these cells, immature to full-fledged leukocytes, do not cope with their main task, which causes a lack of immune defense and resistance to inflammation, allergic agents, with all the ensuing consequences.

Phases

The development of chronic myeloid leukemia occurs in three successive phases.

  • The phase is chronic. This stage lasts about 3.5-4 years. Usually it is with her that most patients get to a specialist. The chronic phase is characterized by constancy, because patients have the minimum possible set of symptomatic manifestations. They are so insignificant that patients at times do not attach any importance to them at all. A similar stage can be detected by an accidental blood test.
  • Accelerating phase. It is characterized by the activation of pathological processes and a rapid increase in immature leukocytes in the blood. The duration of the acceleration period is a year and a half. If the treatment process is chosen adequately and started on time, then the probability of the return of the pathological process to the chronic phase increases.
  • Blast crisis or terminal phase. This is the stage of exacerbation, it lasts no more than six months and ends lethally. It is characterized by an almost absolute replacement of red bone marrow cells with abnormal malignant clones.

In general, the pathology is characterized by a leukemic scenario of development.

Symptoms

The clinical picture of myeloid leukemia varies according to the phase of the pathology. But general symptoms can also be identified.

Patients note causeless weight loss, lethargy, lack of appetite. In the course of the development of the disease, there is a characteristic enlargement of the liver, spleen, symptoms of hemorrhagic origin. Patients tend to bleed, the skin turns pale, bone pains, and nocturnal sweating are disturbing.

chronic stage

For this stage of chronic myeloid leukemia, the following manifestations are typical:

  1. Mild symptoms characteristic of chronic fatigue. General well-being worsens, impotence, weight loss worries;
  2. Due to the increase in the volume of the spleen, the patient notes rapid satiety when eating, pain often occurs in the left abdomen;
  3. In exceptional cases, there are rare symptoms associated with thrombus formation or blood thinning, headaches, impaired memory and attention, visual disturbances, shortness of breath, myocardial infarction.
  4. Men in this phase may develop an excessively long, painful erection or a priapic syndrome.

Accelerative

The accelerative stage is characterized by a sharp increase in the severity of pathological symptoms. Anemia progresses rapidly, and the therapeutic effect of drugs of the cytostatic group decreases markedly.

Laboratory diagnostics of blood shows a rapid increase in leukocyte cells.

Terminal

The blast crisis phase of chronic myeloid leukemia is characterized by a general deterioration in the clinical picture:

  • The patient has pronounced febrile symptoms, but without infectious etiology. The temperature can rise to 39 ° C, causing a feeling of intense trembling;
  • Intensively manifested hemorrhagic symptoms due to bleeding through the skin, intestinal membranes, mucous tissues, etc.;
  • Severe weakness bordering on exhaustion;
  • The spleen reaches an incredible size and is easily palpated, which is accompanied by heaviness and pain in the abdomen on the left.

The terminal stage usually ends lethally.

Diagnostic methods

Diagnosis of this form is managed by a hematologist. It is he who conducts the examination and prescribes laboratory, ultrasound diagnostics of the abdomen. Additionally, a bone marrow puncture or biopsy, biochemistry and cytochemical studies, cytogenetic analysis are performed.

blood picture

For chronic myeloid leukemia, the following blood picture is typical:

  • At the chronic stage, the proportion of myeloblasts in the bone marrow fluid or blood is about 10-19%, and basophils - more than 20%;
  • At the terminal stage, lymphoblasts and myeloblasts exceed the 20% threshold. When conducting a biopsy study of the bone marrow fluid, large accumulations of blasts are found.

Treatment

The therapeutic process for the treatment of chronic myeloid leukemia consists of the following areas:

  1. Chemotherapy;
  2. bone marrow transplant;
  3. Irradiation;
  4. Leukopheresis;
  5. Spleenectomy

Chemotherapeutic treatment involves the use of traditional drugs such as Myelosan, Cytosar, Hydroxyurea, etc. The latest drugs of the latest generation are also used - Sprycel or Glivec. The use of medicines based on hydroxyurea, Interferon-α, etc. is also indicated.

There is an option for a complete recovery of the patient, which can only be achieved through bone marrow transplantation. To do this, a donor is selected among relatives, although donation of strangers is also possible.

After transplantation, the patient has no immune protection, so he is in the hospital until the donor cells take root. Gradually, bone marrow activity returns to normal and the patient recovers.

If chemotherapy is not effective, radiation is used. This procedure is based on the use of gamma rays, which affect the area of ​​the spleen. The aim of this treatment is to stop the growth or destruction of abnormal cells.

In exceptional situations, removal of the spleen is indicated. Such an intervention is carried out mainly in the phase of the blast crisis. As a result, the general course of the pathology improves significantly, and the effectiveness of drug treatment increases.

When the leukocyte level reaches excessively high levels, leukopheresis is performed. This procedure is almost identical to plasmapheresis blood purification. Often, leukopheresis is included in complex therapy with medications.

Life expectancy forecast

The majority of patients die in the accelerating and terminal stages of the pathological process. About 7-10% die after being diagnosed with myelogenous leukemia in the first 24 months. And after a blast crisis, survival can last about 4-6 months.

If it is possible to achieve remission, then the patient can live after the terminal stage for about a year.

A detailed video about the diagnosis and treatment of chronic myelogenous leukemia: