Acute myeloid leukemia (the most commonly used abbreviation is AML) is a malignant pathology of the blood and bone marrow, characterized by the uncontrolled production of immature (blast) myelocytic cells - red blood cells, granulocytes, neutrophilic leukocytes, platelets. The disease is diagnosed in cases where more than 20% of immature cells are detected in the myelogram. Its characteristic feature is its rapid course: within a few months, immature cells begin to predominate in the composition of the blood, which is why it ceases to perform its main functions. The patient's condition deteriorates sharply, and in the absence of treatment, the prognosis is extremely unfavorable.
Kinds
Oncohematologists distinguish several dozen types of acute myeloid leukemia, which are grouped according to similar characteristics:
- with typical genetic mutations;
- with dysplasia that causes cell changes;
- arising as a result of treatment of other diseases;
- with the proliferation of myeloid germ against the background of Down syndrome;
- myeloid sarcomas;
- dendritic cell plasmacytoid tumors.
Depending on the form of the disease, the doctor chooses treatment tactics, since the duration of remissions and the overall prognosis for different types have serious differences.
Prognosis and prevention of chronic myeloid leukemia
There are no specific methods of prevention, since the reasons for the formation of mutations that cause chronic myeloid leukemia are unknown. As for the prognosis, it all depends on the patient’s age, response to treatment and the possibility of allogeneic transplantation. In general, the situation is quite favorable and allows us to hope for a life expectancy comparable to general population indicators. In some patients, it is possible to achieve stable remission and avoid lifelong use of TKIs with regular follow-up.
At the Euroonco clinic, the treatment of myeloid leukemia complies with all modern treatment protocols. In difficult cases, the decision is made by a council of specialists, and when remission is achieved, we pay special attention to regular monitoring of the patient, which also allows us to improve treatment results.
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Symptoms
In the initial stage of acute myeloid leukemia, there are practically no symptoms; as the disease progresses, the patient develops:
- pale skin and other signs of anemia;
- bleeding from the nose and gums, numerous bruises on the skin;
- elevated temperature within 37-38 degrees (subfebrile);
- profuse sweating at night;
- rash on the skin in the form of small reddish spots;
- shortness of breath even after light exertion;
- constant pain in bones and joints;
- frequent colds and other infections;
- enlarged lymph nodes;
- sudden weight loss for no apparent reason.
Causes of myeloid leukemia
The reasons for the development of CML is the exchange of sections of chromosomes 9 and 22. The new chromosome that forms in this case is called the Philadelphia or Ph+ chromosome. The Philadelphia chromosome was discovered in 1960 by Peter Nowell and David Hungerford in Philadelphia. It got its name from the place where the discoverers worked. The Philadelphia chromosome (Ph+) contains an abnormal gene called BCR-ABL. This gene is responsible for the formation of the abnormal protein BCR-ABL, which causes excessive production of abnormal white blood cells.
Causes and risk factors
It is currently unknown what exactly influences induce blood germs to malignant mutations, but oncologists have well studied the factors that contribute to the development of acute myeloid leukemia.
- Exposure to carcinogens. Certain chemical compounds can induce cells to change. These are a number of products formed during tobacco smoking and incomplete oxidation of fats, many types of industrial emissions, some medications, etc.
- Radiation. X-rays and other radioactive, ionizing radiation change the hereditary apparatus of living cells.
- Genetic factor. The risk of the disease increases several times for people who have close relatives with leukemia.
- Diseases. Some diseases increase the likelihood of blood cancer. These are Down syndrome, congenital anemia and thrombocytopenia, neurofibromatosis, etc.
General information
Chronic myeloid leukemia is one of the slowly progressive, but very dangerous malignant diseases. During the development of the disease, those cells that, in the normal state of the body, develop into basophils , eosinophils , neutrophils , monocytes , acquire a malignant nature.
Unfortunately, people who develop myeloid leukemia do not usually learn what it is at the very early stages. After all, at first they are worried only by nonspecific signs - weakness, poor appetite, weight loss. As the disease progresses, the spleen and lymph nodes enlarge, and other signs appear. Treatment is with tyrosine kinase inhibitors, and sometimes a stem cell transplant is required. With the right approach to treatment, the patient’s life can be extended and its quality maintained.
Chronic myeloid leukemia, whose ICD-10 code is C92.1, can affect people at any age. But still, most often chronic myeloid leukemia is diagnosed in people 40-60 years old. In most cases - approximately 90% - the disease develops in a chronic form. The disease is not an inherited pathology.
How this disease manifests itself, as well as the most progressive methods of treating it, will be discussed in this article.
Diagnostics
Laboratory tests of blood and bone marrow samples are the main ways to diagnose acute myeloid leukemia. This:
- general peripheral blood test;
- myelogram;
- cytochemical analysis of immature cells;
- immunophenotyping;
- cytogenetic analysis;
- molecular genetic analysis;
- HLA typing;
- blood chemistry;
- Reberg's test;
- coagulogram.
In addition, the patient is prescribed instrumental examinations of internal organs to assess their condition: ultrasound of the abdominal cavity, ECG, chest x-ray. Subsequently, other tests and studies may be prescribed.
Diet
Diet for leukemia (blood cancer)
- Efficacy: no data
- Duration: until recovery/lifelong
- Cost of products: 3000-4600 rubles. in Week
During the treatment period, it is important to adhere to recommendations regarding proper nutrition. A proper diet with sufficient content of substances important for the body will contribute to the effectiveness of treatment of the disease. During the treatment of myeloid leukemia, it is very important to prevent deficiency of iron, cobalt, copper, manganese, nickel, and vitamin C. All these components are important for the restoration of blood cells, as well as for maintaining the optimal general condition of the patient. The following products should be included in the diet:
- protein foods - sea fish and seafood, rabbit and poultry meat, egg yolks, offal, dairy products;
- vegetables - greens, peppers, cabbage, legumes, carrots, pumpkin, corn, zucchini;
- fruits - citrus fruits, pears, berries, apples, plums, peaches, apricots, sea buckthorn;
- cereals;
- bran bread;
- honey.
It is recommended to exclude from the diet:
- lamb and beef, which contain refractory fat;
- coffee, tea and other products containing caffeine;
- products with vinegar - canned food, etc.;
- alcohol;
- hot spices.
It is recommended to reduce the amount of fat consumed.
Treatment
The main treatment method for acute myeloid leukemia is chemotherapy, which is carried out in two stages:
- induction - reducing the number of immature cells to achieve remission;
- consolidation - maximum prolongation of remission, prevention of relapses, elimination of residual effects.
Intensive therapy used during the induction phase allows achieving remission in approximately 70% of clinical cases. But treatment should absolutely not end there, since without a consolidation program, patients almost always experience a relapse after some time.
The consolidation stage, depending on the patient’s condition, includes up to five courses of chemotherapy. If the risk of relapse is high enough, a bone marrow and/or hematopoietic stem cell transplant is performed. The best donors are siblings, especially identical twins.
In addition, patients are often prescribed the administration of red blood cells and transfusion therapy (transfusions of blood and its drugs). Treatment tactics are usually chosen according to the patient's age. Remission is considered achieved if the number of myeloid cells in the bone marrow can be reduced to 20% or less.
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The essence of the disease
Chronic myeloid leukemia (chronic myeloid leukemia, chronic myeloid leukemia, CML) is a disease in which there is an excessive formation of granulocytes in the bone marrow and an increased accumulation of both these cells and their precursors in the blood. The word “chronic” in the name of the disease means that the process develops relatively slowly, unlike acute leukemia, and “myeloid” means that the process involves cells of the myeloid (rather than lymphoid) lineage of hematopoiesis.
A characteristic feature of CML is the presence in leukemic cells of the so-called Philadelphia chromosome, a special chromosomal translocation. This translocation is designated as t(9;22) or, in more detail, as t(9;22)(q34;q11) - that is, a certain fragment of chromosome 22 changes places with a fragment of chromosome 9. As a result, a new, so-called chimeric, is formed. a gene (designated BCR-ABL), the “work” of which disrupts the regulation of cell division and maturation.
Chronic myeloid leukemia belongs to the group of myeloproliferative diseases.
Incidence and risk factors
In adults, CML is one of the most common types of leukemia. Every year, 1-2 cases per 100 thousand population are registered. In children, it occurs significantly less frequently than in adults: about 2% of all cases of CML occur in childhood. Men get sick slightly more often than women.
The incidence increases with age and is increased among people exposed to ionizing radiation. Other factors (heredity, nutrition, ecology, bad habits) apparently do not play a significant role.
Signs and symptoms
Unlike acute leukemia, CML develops gradually and is divided into four stages: preclinical, chronic, progressive and blast crisis.
At the initial stage of the disease, the patient may not have any noticeable manifestations, and the disease may be suspected accidentally, based on the results of a general blood test. This is the preclinical stage.
Then symptoms such as shortness of breath, fatigue, pallor, loss of appetite and weight, night sweats, and a feeling of heaviness in the left side due to an enlarged spleen appear and slowly increase. Fever and joint pain may occur due to the accumulation of blast cells. The phase of the disease in which the symptoms are not very pronounced and develop slowly is called chronic.
In most patients, the chronic phase after some time - usually after several years - passes into the acceleration phase, or progressive. The number of blast cells and mature granulocytes increases. The patient feels noticeable weakness, pain in the bones and an enlarged spleen; the liver also enlarges.
The most severe stage in the development of the disease is blast crisis, in which the content of blast cells is sharply increased and CML in its manifestations becomes similar to aggressive acute leukemia. Patients may experience high fever, bleeding, bone pain, difficult-to-treat infections, and leukemic skin lesions (leukemids). In rare cases, an enlarged spleen may rupture. Blast crisis is a life-threatening and difficult to treat condition.
Diagnostics
Often, CML is detected before any clinical signs appear, simply by an increased number of white blood cells (granulocytes) in a routine blood test. A characteristic feature of CML is an increase in the number of not only neutrophils, but also eosinophils and basophils. Mild to moderate anemia is common; platelet levels vary and may be elevated in some cases.
If CML is suspected, a bone marrow puncture is performed. The basis for diagnosing CML is the detection of the Philadelphia chromosome in cells. It can be done using cytogenetic or molecular genetic testing.
The Philadelphia chromosome can occur not only in CML, but also in some cases of acute lymphoblastic leukemia. Therefore, the diagnosis of CML is made based not only on its presence, but also on other clinical and laboratory manifestations described above.
Treatment
For the treatment of CML in the chronic phase, a number of drugs have traditionally been used that inhibit the progression of the disease, although they do not lead to a cure. Thus, busulfan and hydroxyurea make it possible to control the level of white blood cells for some time, and the use of alpha interferon (sometimes in combination with cytarabine), if successful, significantly slows down the progression of the disease. These drugs have retained some clinical significance to this day, but now there are much more effective targeted drugs.
A specific agent that allows one to specifically “neutralize” the result of genetic damage in cells in CML is imatinib (Gleevec and others); This drug is significantly more effective than earlier drugs and is much better tolerated. Imatinib can dramatically increase the duration and improve the quality of life of patients. Most patients must take imatinib continuously from the moment of diagnosis: discontinuation of treatment is associated with a risk of relapse, even if clinical and hematological remission has already been achieved.
Treatment with imatinib is carried out on an outpatient basis, the medicine is taken in tablet form. The response to treatment is assessed at several levels: hematological (normalization of clinical blood test), cytogenetic (disappearance or sharp decrease in the number of cells, where the Philadelphia chromosome is detected by cytogenetic analysis) and molecular genetic (disappearance or sharp decrease in the number of cells, where polymerase chain reaction succeeds in detecting the chimeric BCR-ABL gene).
Imanitib is the basis of modern therapy for CML. Potent new drugs are also constantly being developed for patients intolerant or unresponsive to imatinib therapy. Currently, there are newer generation drugs dasatinib (Sprycel) and nilotinib (Tasigna), which can help a significant proportion of these patients.
Unfortunately, as already mentioned, during therapy with imatinib and other drugs, some cells with genetic damage may remain in the bone marrow (minimal residual disease), which means that a complete cure is not achieved. Therefore, in young patients with CML, if a compatible donor is available, bone marrow transplantation is sometimes indicated, although it is now rarely used for this diagnosis.
Forecast
The prognosis for CML depends on the patient's age, the number of blast cells, response to therapy and other factors. In general, new drugs, such as imatinib, allow for many years (there have been no very long observations yet, since these drugs became widespread only at the beginning of the 21st century) to increase the life expectancy of patients with a significant increase in its quality.
Allogeneic bone marrow transplantation is associated with significant risks and is now rarely used for CML, but if successful, complete recovery occurs.
List of sources
- Abdulkadyrov K.M., Bessmeltsev S.S., Rukavitsyn O.A. Chronic myeloid leukemia. – St. Petersburg: “Special Literature”, 1998.- 464 p.
- Quality of life of patients with chronic myeloid leukemia / M. I. Savelyeva, I. N. Samarina, O. S. Samoilova, etc. // Kremlin. medicine. Wedge. Vestn. – 2012. – No. 1. – P. 60-64.
- Savva N.N. Innovative approaches to the treatment of chronic myeloid leukemia in children / N.N. Savva // Republican Scientific and Practical Center of Pediatric Oncology and Hematology of the Ministry of Health of the Republic of Belarus. Medical news. - 2010. - No. 5-6. — P.12-17.
- Smirnova, O. V. Chronic myeloid leukemia - clinical and immunological features in adult patients / O. V. Smirnova // Acta Biomedica Scientifica. – 2012. – No. 3-2. – pp. 185-189.
