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 becomes the cause of impaired 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 disease code C92. It can take 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 function of healthy cells in the body is based on the information that chromosomes contain. When a particular cell divides, it creates a new copy of DNA in the chromosomes. If such a division process is disrupted, mutating genes can be formed, which affect the development of oncological pathologies.
The human body has 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. In case of disruption of the activity of such genes, the degeneration of healthy cells into oncological cells and the exclusion of suppressors from this process is observed.
Modern medicine does not have enough specific information about why chronic myeloid leukemia, including acute, develops. This issue is under study. There are suggestions that some predisposing factors influence the development of the disease:
- 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 says that most of them were susceptible to the development of chronic myeloid leukemia.
- Viral damage to the body, as well as electromagnetic rays and chemicals affecting the body. Such a factor as a potential cause of the development of the disease is still being considered by researchers today.
- Hereditary predisposition... People with congenital chromosomal abnormalities are at an increased risk of myeloid leukemia. In most cases, these are individuals who have been diagnosed with Down syndrome or Klinefelter's syndrome.
- Treatment of tumor-like neoplasms certain medicines of the type of cytostatics in combination with radiation.
All such predisposing factors cause structural damage to the 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, an uncontrolled growth of abnormal cells is observed, by analogy with cancer cells.
Stages of development of the disease
Most people (about 80%) go to the hospital at the time when the disease becomes chronic. At this time, there are mild symptoms of myeloid leukemia, which are often confused with the usual 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, myeloid leukemia is diagnosed quite by accident, by conducting a blood test to identify another pathology in the body.
Chronic myeloid leukemia can be accompanied by complications in the form of an increase in the overall temperature to high rates, pain in the left hypochondrium, etc. In the presence of complications, this form of the disease develops over 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 is added, which aggravates the general condition of the patient: anemia develops, the spleen enlarges, and the drugs used in treatment do not bring the same effectiveness as at the initial stage of the development of the disease. 
If you do not start treatment at the stage of acceleration, 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 a 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 can be called:
- pronounced general malaise;
- weight loss;
- decreased or complete loss of appetite (depending on the stage of the disease);
- the spleen and liver increase in chronic myeloid leukemia;
- blanching of the skin;
- bone pain syndrome;
- increased sweating.
If we consider the clinic of the disease, taking into account its stage, it looks like this:
- Chronic: rapid satiety during a meal, 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.
- Acceleration stage... At this stage, progressive anemia develops, general pathological symptoms increase in intensity, pathological leukocyte cells are at an increased level in the blood.
- Terminal. The general condition of the patient deteriorates to critical levels. A febrile syndrome occurs, the general temperature rises to the maximum mark. Also, the development of terminal myelosis is characterized by bleeding through the mucous membranes, skin, intestines. Due to the enlargement of 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, the following are prescribed:
- Complete blood count... 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 development 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.
- Biochemical analysis... Diagnostics shows an increase in the amount of uric acid in the body.
- Sternal puncture of bone substance... Megakaryocytes are exceeded in their content level, as well as granulocytic cells of young forms.
At the stage of acceleration, it is necessary to carry out the following diagnostic measures:
At the terminal stage, pathology can be identified by conducting:
- Complete blood count, which helps to detect a critical decrease in the volume of erythrocytes, platelets and hemoglobin, an increase in the volume of basophils up to 20%. Leukocytosis reaches 500-1000 * 109 / l.
- Sternal puncture, which helps to identify a critical increase in the content of malignant cells in the medulla, as well as basophils and eosinophils.
- Cytogenetic analysis, which helps to identify the presence of the Philadelphia chromosome in the body.
How to treat a 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 absent altogether, adherence to the correct diet, intake of vitamin preparations, and general strengthening procedures are prescribed. In this case, systematic observation by the attending physician is required.
If pronounced symptoms have joined, cytostatic drugs are prescribed that block the growth of pathological cells. 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 transplants and blood transfusions are performed. Sometimes such treatment helps to permanently save a person from the disease. The only condition is full 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 the help of which it is possible to induce hematological remission of pathology. The substances included in the preparation 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.
- malignant myeloproliferative disease, characterized by a predominant lesion of the granulocytic lineage. It can be asymptomatic for a long time. It is manifested by a tendency to subfebrile condition, a feeling of fullness in the abdomen, frequent infections and an enlarged spleen. Anemia and changes in platelet levels are observed, accompanied by weakness, pallor and increased bleeding. In the final stage, fever, lymphadenopathy and skin rash develop. The diagnosis is established taking into account the history, clinical picture and laboratory data. Treatment - chemotherapy, radiotherapy, bone marrow transplant.
General information
Chronic myeloid leukemia is an oncological disease resulting from chromosomal mutation with damage to pluripotent stem cells and subsequent uncontrolled proliferation of mature granulocytes. It accounts for 15% of the total number of hematological malignancies in adults and 9% of the total number of leukemias in all age groups. Usually develops after 30 years, the peak incidence of chronic myeloid leukemia occurs at the age of 45-55 years. Children under 10 years old suffer extremely rarely.
Chronic myeloid leukemia is equally common in women and men. Due to the asymptomatic or asymptomatic course, it can become an accidental finding when examining a blood test taken in connection with another disease or during a routine examination. In some patients, chronic myeloid leukemia is detected at the final stages, which limits the possibilities of therapy and worsens survival rates. Treatment is carried out by specialists in the field of oncology and hematology.
Etiology and pathogenesis of chronic myeloid leukemia
Chronic myeloid leukemia is considered the first disease in which a connection between the development of pathology and a certain genetic disorder has been reliably established. In 95% of cases, the confirmed cause of chronic myeloid leukemia is a chromosomal translocation known as the "Philadelphia chromosome." The essence of translocation is the interchange of regions 9 and 22 chromosomes. As a result of this replacement, a stable open reading frame is formed. Frame formation accelerates cell division and inhibits DNA repair, which increases the likelihood of other genetic abnormalities.
Among the possible factors contributing to the appearance of the Philadelphia chromosome in patients with chronic myeloid leukemia, ionizing radiation and contact with certain chemical compounds are called. The result of the mutation is the increased proliferation of pluripotent stem cells. In chronic myeloid leukemia, mainly mature granulocytes proliferate, but the abnormal clone also includes other blood cells: erythrocytes, monocytes, megakaryocytes, less often B- and T-lymphocytes. In this case, normal hematopoietic cells do not disappear and, after suppression of the abnormal clone, can serve as the basis for normal proliferation of blood cells.
Chronic myeloid leukemia is characterized by a staged course. In the first, chronic (inactive) phase, there is a gradual aggravation of pathological changes while maintaining a satisfactory general condition. In the second phase of chronic myeloid leukemia - the acceleration phase, changes become apparent, progressive anemia and thrombocytopenia develop. The final stage of chronic myeloid leukemia is a blast crisis, accompanied by rapid extramedullary proliferation of blast cells. The source of blasts is the lymph nodes, bones, skin, central nervous system, etc. In the phase of the blast crisis, the state of the patient with chronic myeloid leukemia sharply deteriorates, severe complications develop, ending in the death of the patient. In some patients, the acceleration phase is absent, the chronic phase is immediately replaced by a blast crisis.
Chronic myeloid leukemia symptoms
The clinical picture is determined by the stage of the disease. The chronic phase lasts on average 2-3 years, in some cases - up to 10 years. This phase of chronic myeloid leukemia is characterized by an asymptomatic course or the gradual appearance of "mild" symptoms: weakness, some malaise, decreased ability to work and a feeling of fullness in the abdomen. An objective examination of a patient with chronic myeloid leukemia may reveal an enlargement of the spleen. According to blood tests, an increase in the number of granulocytes up to 50-200 thousand / μl with an asymptomatic course of the disease and up to 200-1000 thousand / μl with "mild" symptoms is revealed.
In the initial stages of chronic myeloid leukemia, a slight decrease in hemoglobin levels is possible. Subsequently, normochromic normocytic anemia develops. When examining a blood smear of patients with chronic myeloid leukemia, a predominance of young forms of granulocytes is noted: myelocytes, promyelocytes, myeloblasts. There are deviations from the normal level of grain in one direction or another (abundant or very scarce). The cytoplasm of cells is immature, basophilic. Anisocytosis is determined. If untreated, the chronic phase enters the acceleration phase.
The beginning of the phase of acceleration of chronic myeloid leukemia can be evidenced by both a change in laboratory parameters and a worsening of the patient's condition. Possibly an increase in weakness, an enlarged liver, and a progressive enlargement of the spleen. In patients with chronic myeloid leukemia, clinical signs of anemia and thrombocytopenia or trobocytosis are revealed: pallor, fatigue, dizziness, petechiae, hemorrhages, increased bleeding. Despite the ongoing treatment, the number of leukocytes in the blood of patients with chronic myeloid leukemia is gradually increasing. At the same time, there is an increase in the level of metamyelocytes and myelocytes, the appearance of single blast cells is possible.
The blast crisis is accompanied by a sharp deterioration in the patient's condition with chronic myeloid leukemia. New chromosomal abnormalities appear, a monoclonal neoplasm is transformed into a polyclonal one. An increase in cellular atypism is noted with the suppression of normal hematopoiesis germs. Pronounced anemia and thrombocytopenia are observed. The total number of blasts and promyelocytes in the peripheral blood is more than 30%, in the bone marrow - more than 50%. Patients with chronic myeloid leukemia lose weight and appetite. Extramedullary foci of immature cells (chloromas) appear. Bleeding and severe infectious complications develop.
Diagnostics of the chronic myeloid leukemia
The diagnosis is established on the basis of the clinical picture and the results of laboratory tests. The first suspicion of chronic myeloid leukemia often arises when the level of granulocytes in the general blood test is increased, prescribed as a routine examination or examination in connection with another disease. To clarify the diagnosis, the data of histological examination of the material obtained by sternal puncture of the bone marrow can be used, however, the final diagnosis of chronic myeloid leukemia is made when the Philadelphia chromosome is detected using PCR, fluorescent hybridization or cytogenetic research.
The question of the possibility of diagnosing chronic myeloid leukemia in the absence of the Philadelphia chromosome remains controversial. Many researchers believe that such cases can be explained by complex chromosomal abnormalities, due to which the identification of this translocation becomes difficult. In some cases, the Philadelphia chromosome can be detected using reverse transcription PCR. With negative research results and an atypical course of the disease, they usually speak not of chronic myeloid leukemia, but of undifferentiated myeloproliferative / myelodysplastic disorder.
Treatment of chronic myeloid leukemia
Treatment tactics are determined depending on the phase of the disease and the severity of clinical manifestations. In the chronic phase, with an asymptomatic course and poorly expressed laboratory changes, they are limited to general strengthening measures. Patients with chronic myeloid leukemia are advised to observe the regime of work and rest, eat food rich in vitamins, etc. When the level of leukocytes increases, busulfan is used. After normalization of laboratory parameters and reduction of the spleen, patients with chronic myeloid leukemia are prescribed supportive therapy or a course of treatment with busulfan. Radiation therapy is commonly used for leukocytosis in combination with splenomegaly. With a decrease in the level of leukocytes, a pause is made for at least a month, and then they switch to maintenance therapy with busulfan.
In the progressive phase of chronic myeloid leukemia, it is possible to use one chemotherapy drug or polychemotherapy. Apply mitobronitol, hexaphosphamide or chloroethylaminouracil. As in the chronic phase, intensive therapy is carried out until laboratory parameters are stabilized, and subsequently they are switched to maintenance doses. Polychemotherapy courses for chronic myeloid leukemia are repeated 3-4 times a year. With blast crises, treatment with hydroxycarbamide is carried out. If therapy is ineffective, leukocytapheresis is used. With severe thrombocytopenia, anemia, transfusions of thromboconcentrate and erythrocyte mass are performed. With chloromas, radiotherapy is prescribed.
Bone marrow transplant is performed in the first phase of chronic myeloid leukemia. Long-term remission can be achieved in 70% of patients. If indicated, splenectomy is performed. Emergency splenectomy is indicated in case of rupture or threat of rupture of the spleen, planned - with hemolytic crises, "wandering" spleen, recurrent perisplenitis and severe splenomegaly, accompanied by dysfunction of the abdominal organs.
Chronic myeloid leukemia prognosis
The prognosis for chronic myeloid leukemia depends on many factors, the determining one of which is the moment of initiation of treatment (in the chronic phase, the activation phase, or during the blast crisis). As unfavorable prognostic signs of chronic myeloid leukemia, consider a significant increase in the liver and spleen (the liver protrudes from under the edge of the costal arch by 6 cm or more, the spleen - by 15 cm or more), leukocytosis over 100x10 9 / l, thrombocytopenia less than 150x10 9 / l , thrombocytosis more than 500x10 9 / l, an increase in the level of blast cells in the peripheral blood up to 1% or more, an increase in the total level of promyelocytes and blast cells in the peripheral blood up to 30% or more.
The likelihood of an unfavorable outcome in chronic myeloid leukemia increases as the number of symptoms increases. Infectious complications or severe hemorrhages become the cause of death. The average life expectancy of patients with chronic myeloid leukemia is 2.5 years, however, with timely initiation of therapy and a favorable course of the disease, this indicator can increase to several decades.
Chronic myeloid leukemia- tumor blood disease. It is characterized by uncontrolled growth and reproduction of all growth blood cells, while young malignant cells are able to mature to mature forms.
Chronic myeloid leukemia (synonym - chronic myeloid leukemia) - tumor blood disease. Its development is associated with changes in one of the chromosomes and the appearance chimeric ("Stitched" from different fragments) a gene that disrupts hematopoiesis in the red bone marrow.
During chronic myeloid leukemia, the content of a special type of leukocytes in the blood rises - granulocytes ... They are formed in the red bone marrow in huge quantities and are released into the blood, not 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 myeloid leukemia:
- Every fifth tumor of the blood is chronic myeloid leukemia.
- Among all blood tumors, chronic myeloid leukemia ranks third in North America and Europe, in Japan - second.
- Chronic myeloid leukemia affects 1 in 100,000 people worldwide 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.
The causes of chronic myeloid leukemia
The causes of chromosomal abnormalities leading to chronic myeloid leukemia are still poorly understood.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 displace all the others and occupy the bulk of the red bone marrow. The vicious gene has three main effects:
- Cells proliferate uncontrollably, like cancerous 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 are in this phase (about 85%). The average duration is 3-4 years (depending on how timely and correct treatment is started). This is the stage of relative stability. The patient is worried about minimal symptoms, which he may not pay attention to. Sometimes doctors detect the chronic phase of myeloid leukemia by chance, during a general blood test.
- Acceleration phase... During this phase, the pathological process is activated. The number of immature white blood cells in the blood begins to build up rapidly. The phase of acceleration is, as it were, transitional from chronic to the last, third.
- Terminal phase... The final stage of the disease. It occurs with an increase in changes in chromosomes. Red bone marrow is almost completely replaced by malignant cells. During the terminal stage, the patient dies.
Manifestations of chronic myeloid leukemia
Chronic phase symptoms:
More rare symptoms of the chronic phase of myeloid leukemia
:- Signs associated with impaired platelet and white blood cell function : various bleeding or, on the contrary, 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, loss of memory, attention, etc.), myocardial infarction, visual impairment, shortness of breath.
Acceleration Phase Symptoms
In the acceleration phase, the signs of the chronic stage increase. Sometimes it is at this time that the first signs of the disease appear, which force the patient to visit the doctor for the first time.Symptoms of the terminal stage of chronic myeloid leukemia:
- Severe weakness , a significant deterioration in general health.
- Prolonged aching pain in joints and bones ... They can sometimes be very powerful. This is due to the proliferation 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 platelet count and decreased blood clotting.
- Rapid enlargement of the spleen : the abdomen 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 see if I have symptoms of chronic myeloid leukemia?
A hematologist is involved in the treatment of blood diseases of a tumor nature. Many patients initially turn to a therapist, who then sends them for a consultation with a hematologist.
Examination in the doctor's office
Reception in the hematologist's office is carried out as follows:- Patient questioning ... The doctor finds out the patient's complaints, specifies the time of their occurrence, asks other necessary questions.
- Feeling of the lymph nodes : submandibular, cervical, axillary, supraclavicular and subclavian, elbow, inguinal, popliteal.
- Feeling the belly to determine the enlargement of the liver and spleen. The liver is palpated under the right rib in the supine position. The spleen is on the left side of the abdomen.
When can a doctor suspect a patient has chronic myeloid leukemia?
The symptoms of chronic myeloid leukemia, especially in the initial stages, are nonspecific - they can occur in many other diseases. Therefore, the doctor cannot suggest a diagnosis only on the basis of examination and patient complaints. Usually, the suspicion arises from one of two studies:- 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 performed for suspected chronic myeloid leukemia??
| Study title | Description | What does it reveal? |
| General blood analysis | Routine clinical research is performed if 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 vein in the morning.
| The result depends on the phase of the disease. Chronic phase:
|
| 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 examination, a small fragment of it is obtained using a special needle and sent to the laboratory for examination under a microscope. Carrying out the procedure:
| 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 research | When special dyes are added to blood and red bone marrow samples, some substances may react with them. This is the basis for conducting a 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:
|
| 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 to 30 days. The laboratory uses special modern tests, during which different parts of the DNA molecule are identified.
| In chronic myeloid leukemia, during a cytogenetic study, a chromosomal disorder is detected, which was named philadelphia chromosome
. In the cells of patients, chromosome number 22 is shortened. The lost site joins chromosome number 9. In turn, a fragment of chromosome # 9 joins chromosome # 22. There is, as it were, an exchange, as a result of which genes begin to work incorrectly. The result is myeloid leukemia. Also, other pathological changes on the part of chromosome number 22 are detected. By their nature, one can partially judge the prognosis of the disease. |
| Ultrasound of the abdominal organs. | Ultrasound is used in patients with myeloid leukemia to detect enlarged liver and spleen. An ultrasound scan helps distinguish leukemia from other diseases. |  |
Laboratory indicators
General blood analysis- 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 the 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
The manifestation of symptoms depends on the phase of the disease.Phase I (chronic)
- Long time without symptoms (3 months to 2 years)
- Severity in the left hypochondrium (due to the enlargement of 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, overly prolonged erection.
These symptoms are precursors of a serious condition (blast crisis), appear 6-12 months before its onset.
- The effectiveness of drugs (cytostatics) decreases
- Anemia develops
- The percentage of blast cells in the blood increases
- General condition worsens
- The spleen is enlarged
- Symptoms consistent with the clinical picture in acute leukemia ( see Acute lymphoid leiosis).
How is myeloid leukemia treated?
Purpose of treatment reduce the growth of tumor cells and shrink the spleen.Treatment of the disease should be started as soon as the diagnosis is made. The prognosis largely depends on the quality and timeliness of therapy.
Treatment includes various methods: chemotherapy, radiation therapy, spleen removal, bone marrow transplant.
Medication treatment
Chemotherapy- Classic drugs: Mielosan (Mileran, Busulfan), Hydroxyurea (Hydrea, Litalir), Cytosar, 6-mercaptopurni, alpha interferon.
- New drugs: Gleevec, Sprycel.
| Name | Description |
Hydroxyurea preparations:
| How does the drug work?: Hydroxyurea is a chemical compound that can inhibit the synthesis of DNA molecules in tumor cells. When can they appoint: In chronic myeloid leukemia, accompanied by a significant increase in the number of leukocytes in the blood. How is prescribed: The drug is available in capsule form. The doctor prescribes their reception to the patient in accordance with the selected dosage regimen. Possible side effects:
|
| Glivec (imatinib mesylate) | How does the drug work?: The drug inhibits the growth of tumor cells and enhances the process of their natural death. When can they appoint:
The drug is available in tablet form. The scheme of application and dosage is chosen by the attending physician. Possible side effects: It is difficult to assess the side effects of the drug, since the patients who take it usually already have pronounced disorders on the side of different organs. According to statistics, the drug has to be canceled due to complications quite rarely:
|
| Interferon alpha | How does the drug work?: Interferon alpha increases the body's immune forces and inhibits the growth of cancer cells. When appoint: Usually, interferon-alpha is used for long-term maintenance therapy after the number of leukocytes in the blood has returned to normal. How is prescribed: The drug is used in the form of injection solutions, injected intramuscularly. Possible side effects: Interferon has a fairly large number of side effects, and this is associated with certain difficulties in its use. With the correct prescription of the drug and constant monitoring of the patient's condition, the risk of undesirable effects can be minimized:
|
Bone marrow transplantation
A bone marrow transplant makes it possible for patients with chronic myeloid leukemia to recover completely. The efficiency of transplantation is higher in the chronic phase of the disease, in other phases it is much lower. Red bone marrow transplantation is the most effective treatment for chronic myeloid leukemia. More than half of the transplant patients experience lasting improvement over 5 years or longer.
Most often, recovery occurs when the red bone marrow is transplanted to a patient younger than 50 years old in the chronic phase of the disease.
Stages of red bone marrow transplantation:
- Donor search and preparation... The best donor of stem cells of red bone marrow is a close relative of the patient: a twin, brother, sister. If there are no close relatives, or they are not suitable, they look for a donor. A number of tests are performed to make sure that the donor material has taken root in the patient's body. Today, developed countries have created large donor banks, which contain tens of thousands of donor samples. This gives you the chance to find the right stem cells faster.
- Patient preparation... This stage usually lasts from a week to 10 days. Radiation therapy and chemotherapy are performed to destroy as many tumor cells as possible, to prevent the rejection of donor cells.
- Red bone marrow transplant itself... 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 blood. They circulate in the bloodstream for some time, and then settle in the bone marrow, take root there and begin to work. To prevent the rejection of 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 to 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 well-being begins to improve.
- Recovery... Over the course of several months or years, the function of the red bone marrow 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, about a year after the bone marrow transplant, vaccinations are given.
Radiation therapy
It is carried out in cases of lack of effect from chemotherapy and with an enlarged spleen after taking medications (cytostatics). Method of choice for the development of a local tumor (granulocytic sarcoma). In what phase of the disease is radiation therapy applied?
Radiation therapy is used in the advanced stage of chronic myeloid leukemia, which is characterized by the following symptoms:
- Significant growth of tumor tissue in the red bone marrow.
- The growth of tumor cells in long 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 the radiation regime are determined by the attending physician.
Removal of the spleen (splenectomy)
Removal of the spleen is rarely used for limited indications (spleen infarction, thrombocytopenia, severe abdominal discomfort).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?
- Spleen ruptured.
- Threatened rupture of the spleen.
- A significant increase in the size of the organ, which leads to severe discomfort.
Cleaning the blood from excess leukocytes (leukapheresis)
With the development of a blast crisis, the treatment will be the same as in acute leukemia (see acute lymphocytic leukemia).
Leukocytapheresis - a treatment procedure reminiscent of plasmapheresis (blood purification). A certain amount of blood is taken from the patient and passed through a centrifuge, in which it is purified from tumor cells.
In what phase of the disease is leukocytapheresis performed?
Just like radiation therapy, leukocytapheresis is performed during the advanced stage of myeloid leukemia. It is often used in cases where there is no effect from the use of drugs. Sometimes leukocytapheresis complements drug therapy.
Chronic myeloid leukemia is a cancer of the blood, which is characterized by a decrease in the level of leukocytes and the appearance of a large number of immature cells - granulocytes.
According to statistics, the incidence of myeloid leukemia is the same in women and men, most often at the age of 30-40 years.
The causes of chronic myeloid leukemia
Among the main factors provoking a cancer of the blood are:
- Hereditary predisposition - cases of blood cancer are recorded in relatives
- Genetic predisposition - the presence of congenital chromosomal mutations, such as Down syndrome, increases the likelihood of the disease
- Exposure to radiation
- The use of chemotherapy and radiation therapy in the treatment of other cancers can provoke myeloid leukemia
Stages of chronic myeloid leukemia
The development of chronic myeloid leukemia occurs in three successive stages:
Chronic stage
The longest stage, which usually lasts 3-4 years. Most often, it is asymptomatic or with a blurred clinical picture, which does not cause suspicions about the tumor nature of the disease either from doctors or patients. Chronic myeloid leukemia is detected, usually with a random blood test.
Accelerative stage
At this stage, the disease is activated, the level of abnormal blood cells increases at a rapid rate. The duration of the acceleration is about a year.
At this stage, with proper therapy, there is a chance to return leukemia to the chronic stage.
Terminal stage
The most acute stage lasts no more than 6 months and ends fatally. At this stage, blood cells are almost completely replaced by pathological granulocytes.
Chronic myeloid leukemia symptoms
The manifestations of the disease directly depend on the stage.
Chronic stage symptoms:
In most cases, it is asymptomatic. Some patients complain of weakness, increased fatigue, but, as a rule, do not attach importance to this. At this stage, the disease is detected during the next blood test.
In some cases, there may be weight loss, loss of appetite, increased sweating, especially during a night's sleep.
With an enlarged spleen, pain in the left abdomen may occur, especially after eating.
In rare cases, a tendency to bleed develops due to a decrease in platelet levels. Or, on the contrary, with their increase, blood clots are formed, which is fraught with myocardial infarction, stroke, impaired vision and breathing, headaches.
Symptoms of the accelerated stage:
As a rule, it is at this stage that the first manifestations of the disease are felt. Patients complain of poor health, severe weakness, excessive sweating and pain in joints and bones. Worried about an increase in body temperature, increased bleeding and an increase in the abdomen due to the growth of tumor tissue in the spleen.
Diagnostics of the chronic myeloid leukemia
The oncologist-hematologist deals with the diagnosis of chronic myeloid leukemia.
Blood tests
The main diagnostic method. It can be used not only to diagnose, but also to determine the stage of the pathological process.
At the chronic stage, in the general blood test, an increase in platelets and the appearance of granulocytes are noted against the background of a decrease in the total number of leukocytes.
At the accelerating stage, the share of granulocytes already accounts for 10-19% of leukocytes, the platelet content can be either increased or, on the contrary, decreased.
In the terminal stage, the number of granulocytes increases steadily, and the level of platelets falls.
A biochemical blood test is performed to analyze the functioning of the liver and spleen, which, as a rule, suffer from myeloid leukemia.
Bone marrow biopsy
For this study, the bone marrow is taken with a thin needle, after which the material is sent to the laboratory for detailed analysis.
Most often, the bone marrow is taken from the head of the femur, however, the calcaneus, sternum, and wings of the pelvic bones can be used.
A picture similar to a blood test is observed in the bone marrow - the number of immature leukocytes increases.
Hybridization and PCR
A study such as hybridization is necessary in order to identify an abnormal chromosome, and PCR is an abnormal gene.
Cytochemical research
The essence of the study is that when special dyes are added to blood samples, certain reactions are observed. According to them, the doctor can not only determine the presence of a pathological process, but also carry out differential diagnostics between chronic myeloid leukemia and other variants of blood cancer.
In a cytochemical study in chronic myeloid leukemia, a decrease in alkaline phosphatase is observed.
Cytogenetic studies
This study is based on the study of the patient's genes and chromosomes. For this, blood is taken from the vein, which is sent for a special analysis. The result, as a rule, is ready only after a month.
In chronic myeloid leukemia, the so-called Philadelphia chromosome is found - the culprit for the development of the disease.
Instrumental research methods
Ultrasound, computed and magnetic resonance imaging are necessary for the diagnosis of metastases, the state of the brain and internal organs.
Treatment of chronic myeloid leukemia
Bone marrow transplantation gives a real chance of recovery for patients with chronic myeloid leukemia.
This treatment option consists of several successive stages.
Search for a bone marrow donor. Close relatives are the most suitable donor for transplantation. If a suitable candidate is not found among them, it is necessary to look for such a person in special donor banks.
After it is found, various compatibility tests are carried out to make sure that the donor material will not be aggressively perceived by the patient's body.
Preparing the patient for surgery lasts 1-1.5 weeks. At this time, the patient undergoes chemotherapy and radiation therapy.
Bone marrow transplantation.
During the procedure, a catheter is inserted into the patient's vein, through which stem cells enter the bloodstream. They settle in the bone marrow and after a while begin to work there. To prevent the main complication - rejection - medications are prescribed to suppress the immune system and prevent inflammation.
Decreased immunity. It usually takes about a month from the moment the stem cells are introduced to the start of their work in the patient's body. At this time, under the influence of special drugs, the patient's immunity is reduced, this is necessary to prevent rejection. However, on the other hand, this creates a high risk of infection. The patient should spend this period in a hospital, in a special ward - he is protected from contact with a possible infection. Antifungal and antibacterial agents are prescribed, body temperature is constantly monitored.
Cell engraftment. The patient's well-being gradually begins to improve and return to normal.
Restoration of bone marrow function takes several months. During this entire period, the patient is under the supervision of a doctor.
Chemotherapy
In chronic myeloid leukemia, several groups of drugs are used:
Hydroxyurea preparations that inhibit DNA synthesis in tumor cells. Side effects include digestive disorders and allergies.
Of the modern drugs, protein tyrosine kinase inhibitors are often prescribed. These drugs inhibit the growth of abnormal cells, stimulate their death, and can be used at any stage of the disease. Side effects include cramps, muscle pain, diarrhea, and nausea.
Interferon is prescribed after the normalization of the number of leukocytes in the blood to suppress the formation and growth and restore the patient's own immunity.
Possible side effects include depression, mood swings, weight loss, autoimmune pathologies and neuroses.
Radiation therapy
Radiation therapy for chronic myeloid leukemia is carried out in the absence of the effect of chemotherapy or in preparation for a bone marrow transplant.
Gamma irradiation of the spleen helps to inhibit tumor growth.
Splenectomy
In rare cases, removal of the spleen or, in medical terms, splenectomy may be prescribed. The indications for this are a sharp decrease in platelets or severe abdominal pain, a significant increase in the organ or the threat of rupture.
Leukocytophoresis
A significant increase in white blood cells can lead to serious complications such as microthrombosis and retinal edema. In order to prevent them, your doctor may prescribe leukocytophoresis.
This procedure is similar to a regular blood purification, only in this case tumor cells are removed from it. This improves the patient's condition and prevents complications. Leukocytophoresis can also be used in combination with chemotherapy to improve treatment response.
Chronic myeloid leukemia (chronic myeloid leukemia) is a form of leukemia (leukemia) characterized by unregulated and accelerated proliferation (multiplication 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 of occurrence
The occurrence of CML is associated with a genetic abnormality, which is represented by a chromosomal translocation, manifested by the presence of a Ph "chromosome (Philadelphia chromosome) in the karyotype.
Chronic myeloid leukemia symptoms
In the chronic phase of CML, symptoms may be completely absent or mild. A violation of the general condition is possible - the appearance of malaise and weakness, loss of 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, an increase in the severity of symptoms of the chronic stage is observed. Sometimes it is during this phase that the first clear 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, hemorrhages on the body, rapid weight loss, the appearance of torrential sweats, prolonged pain in the joints and aching bones (in some cases, these pains become very severe). It is also possible to periodically cause an unreasonable increase in body temperature up to 38 - 39⁰C with severe chills. A rapid increase in the size of the spleen is characteristic.
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 have 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) - this is the final stage of chronic myeloid leukemia... This phase proceeds like acute leukemia and is characterized by rapid progression and low survival.
Patient actions
The initial stage of chronic myeloid leukemia usually occur latently. However, there are still some nonspecific signs that make it possible to suspect CML.
The somatic signs 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 nonspecific - 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 the reason for seeking 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, and bone marrow transplants.
Complications
Typical complications of CML are hemorrhagic syndrome, infection, and respiratory tract damage. 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.
