There are many hematological diseases (leukemias) belonging to the group of chronic myeloproliferative diseases.
"Myelo" means "bone marrow," and proliferation means "reproduction." Thus, we are talking about diseases associated with the improper generation of one of the main cellular components of the blood – myelocytes.
Patients with myeloproliferative diseases are at risk of thrombosis and hemorrhagic complications, as well as secondary acute leukemia against the background of the underlying disease and its treatment.
Myeloproliferative diseases are one and a half times more common in men than in women. In clinical practice, cases of diseases are most often described among patients aged 40-60 years. Such diseases are not typical for people under 20 years of age and are extremely rare in children.
Patients may have:
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fatigue;
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anorexia, weight loss;
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stomach discomfort and rapid food saturation caused by splenomegaly (enlarged spleen). It is most common in chronic myeloid leukemia and anogenous myeloid metaplasia;
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disposition to bruises, bleeding, and/or symptoms of thrombosis;
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swelling, joint pain caused by gouty arthritis, which results in hyperuricemia (increased uric acid in the blood);
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tinnitus or impaired consciousness in leukostasis;
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pain in the upper left quadrant of the abdomen and left shoulder as a result of a heart attack or an inflammatory process in the spleen.
Among the clinical symptoms, there may be:
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pallor;
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polythora caused by polycythemia;
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local and/or subcutaneous hemorrhage;
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palpable spleen and/or liver;
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fever attacks are possible, accompanied by painful, mottled purple formations on the body, arms, legs and face.
Various laboratory tests and biopsies can be used to diagnose myeloproliferative diseases.
Laboratory tests:
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general blood test with calculation of leukocyte formula and microscopy of a blood smear;
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determination of the level of alkaline phosphatase (LAP) for the differential diagnosis between myeloid leukemia and other causes of leukocytosis;
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PCR or FISH of peripheral blood to diagnose the bcr-abl mutation, which helps differentiate chronic myeloid leukemia from another myeloproliferative disease;determination of red blood cell mass;determination of plasma uric acid levels;performing PCR to determine the JAK2 mutation in suspected cases of true polycythemia, essential thrombocytopenia, and myelofibrosis.BiopsyBone marrow aspiration and biopsy, along with cytogenetic studies, are performed in most cases, but not in all. Cytogenetic studies reveal the presence or absence of the Philadelphia chromosome and help distinguish myeloproliferative diseases from myelodysplastic syndrome.Histological examination of the bone marrow reveals an increased content of parenchymal cells in most cases of diseases of this kind. Bone marrow myelofibrosis is detected by reticulin staining.Patients with painful enlarged spleen who are resistant to myelosuppressive therapy sometimes receive radiation therapy, but usually they need to have their spleen removed.It is necessary to pay attention to seemingly harmless symptoms, since most patients with such diseases have more severe and potentially dangerous vascular complications (massive thrombosis, bleeding), provoked directly by the disease.Patients with established diagnoses should remain under the supervision of a hematologist for life.
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blood transfusion (hemotransfusion);
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chemotherapy;
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bone marrow transplantation.
Patients whose course of disease is not complicated by infectious diseases, anemia, and hemorrhagic syndrome (bleeding) may be monitored and not receive ongoing treatment (the "watch and wait" approach).
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