Chronic myeloid leukemia
The substrate of chronic myeloid leukemia are usually mature and mature cells of the granulocytic series (metamyelocytes, stab and segmented granulocytes). Disease - one of the most common leukemia in the group occurs in people aged 20 - 60 years only rarely, in old people and children, and takes years.
The clinical picture depends on the stage of the disease.
They distinguish three stages of chronic myeloid leukemia - initial, comprehensive and terminal.
In the initial phase of chronic myeloid leukemia is diagnosed almost by accident and gave a blood analysis, because the symptoms during that time almost completely missing. It is drawn to the constant and unmotivated neutrophilic leukocytosis with left shift profile. An enlargement of the spleen, the discomfort in the upper left quadrant, a feeling of heaviness, especially after eating leads. Leukocytosis increased to 40 to 70 g / liter. An important feature is the increase in the number of hematological eosinophils and basophils with different maturity. Anemia in this period was not observed. Marked thrombocytosis at 600 - 1500 g / liter. Virtually indistinguishable at this stage can not. The disease is usually at the level of generalization of total bone marrow tumor, that is diagnosed in advanced stages.
The expanded phase is associated with the onset of clinical symptoms with the leukemic process is characterized. Patients report fatigue, sweating, low-grade fever, weight loss. Appearance and severity of pain in the left upper quadrant, especially after walking. An objective examination of almost constant feature during this period is the enlargement of the spleen, reaching in some cases quite large. On palpation, the spleen is painless. Half of the patients develop heart attacks the spleen, severe pain manifested in the left upper quadrant radiating to the left, the left shoulder, making a deep breath.
The liver also increased, but the size is adjustable. Functional disorders of the liver only slightly expressed. Hepatitis is dyspeptic complaints, jaundice, enlarged liver, an increase of direct bilirubin in the blood manifested. Lymphadenopathy in the advanced stages of chronic myeloid leukemia is a rare hemorrhagic syndrome is lacking.
Can be observed violations of the cardiovascular system (heart pain, heart rhythm disorders). These changes are due to poisoning of the organism increases anemia. Normochromic anemia is character, often pronounced anisotropy and echinosis. In the leukocyte formula of the entire granulocyte series up to and including myeloblasts. The number of white blood cell count is 250 to 500 g / liter. The duration of this phase without cytostatic therapy is only visible by 1.5 to 2 5 years. The clinical picture of treatment is very different. Nausea for a long time there was satisfactory performance, the number of white blood cells - 10 - was 20 g / L, progressive enlargement of the spleen was observed. Expanded stage patients cytostatics takes 4 to 5 years, sometimes longer.
In the final stage was a significant deterioration in general health status, increased sweating, persistent unexplained fever. There are a lot of pain in the bones and joints. An important feature is the appearance of refractoriness to treatment. Significantly increased the spleen. Increased anemia and thrombocytopenia. With a moderate increase in the number of white blood cells rejuvenated formula by increasing the percentage of immature cells (blasts and promyelocytes, myeloblasts and not differentiable).
Hemorrhagic syndrome, unknown in the developed stage, almost always in the terminal period. Neoplastic process in the terminal phase begins on the bone marrow to spread: there are leukemic infiltration of nerve roots, radicular pain, which is administered by subcutaneous leukemic infiltrates (leykemidy) sarkomny growth in lymph nodes produced. Leukemic infiltration of the mucosa contributes to the development of these hemorrhages and subsequent necrosis. In end-stage patients prone to infectious complications that develop often the cause of death.
The differential diagnosis of chronic myeloid leukemia should primarily with myeloid leukemoid reaction type can be performed (as a result of the reaction to an infection, poisoning, etc.). Blast crisis of chronic myeloid leukemia produces a picture that resembles acute leukemia. In this case in favor of chronic myeloid leukemia showed anamnestic data, marked splenomegaly, the presence of bone marrow Philadelphia chromosome.
The treatment of chronic myeloid leukemia in the developed and the final stage is different.
In the advanced stage of therapy aimed at reducing the mass of tumor cells and aims to save as much as possible replacement for somatic patients and the onset of blast crisis. The main drugs used to treat chronic myeloid leukemia: mielosan (Myleran, busulfan), mielobromol (dibromomannitol) geksofosfamid, dopa, 6-mercaptopurine, radiation, times from 1500 to 2000.
The patient was the removal of bottlenecks, the maximum length of stay in the fresh air, smoking and drinking is recommended. Preferred meat, vegetables and fruits. Excluded (fire) in the sun. Heat, physiological and electro procedures contraindicated. In the case of the reduction of red blood cells gemostimulin indices assigned ferropleks. Prices of vitamin B1, B2, B6, C, PP.
Contraindications for radiation blast crisis, severe anemia and thrombocytopenia.
After achieving the therapeutic effect of the changeover to the maintenance dose. Radiotherapy and cytotoxic drugs should be used against the weekly transfusions of 250 ml of blood and the corresponding single-group Rh accessories.
The treatment of end-stage chronic myeloid leukemia in the presence of blasts in the peripheral blood through a system of acute myeloid leukemia. VAMP, tsampa, AVAMP, TSOAP, the combination of vincristine with prednisone with Cytosar rubomitsinom. The therapy is to prolong the life of the patients in remission at this time is determined, is difficult.
The prognosis of this disease is poor. Life expectancy - 4.5 years, in some patients - 10 to 15 years.
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