Journal of Biomedicine and Practice – Biomeditsina va amaliyot jurnali 9-том 2-сан (2024)
COMPLICATIONS OF PRIMARY MYELOFIBROSIS AND TACTICS OF THEIR TREATMEN
АБДИЕВ Каттабек Махматович
Аннотация
Primary myelofibrosis (PM) is rare disease with newly incidence about 1 : 100 000 inhabitants per year. The names for primary myelofi brosis used before were agnogenic myeloid metaplasia, chronic idiopathic metaplasia, osteomyelofibrosis, subleukemic myelosis. The most frequent complications of the clinical course of PMF may be: tumor intoxication, splenomegaly, anemia, infectious complications, thrombocytopenia and hemorrhagic syndrome, the presence of foci of extramedullary hematopoiesis, thrombosis, blast transformation, uric acid diathesis (secondary gout), secondary hemosiderosis. During the study of this disease, the following results were established. For the first time as an independent nosological form of myeloproliferative disease in 1951, William Dameshek called it idiopathic or agnogenic myeloid metaplasia. Subsequently, the combination of the disease with leukocytosis, splenomegaly and bone marrow fibrosis was described in different countries as primary osteosclerosis/osteomyelofibrosis, agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, osteomyelofibrosis, subleukemic myelosis. When treating the disease, that is, primary myelofibrosis, the use of transfusions of hemocomponents containing red blood cells leads to a rapid improvement in the patient's condition and a decrease in the manifestations of anemic syndrome. At the same time, long-term use of transfusions is due to the lack of active mechanisms for removing iron from the body and the limited ability to store it in the liver, when the number of transfusions exceeds 20-25 doses, with the accumulation of iron in organs and tissues leading to the development of secondary hemosiderosis. Patients may have a high rate of development of hemosiderosis, since against the background of chronic anemia, the absorption of iron from the gastrointestinal tract increases. It has been proven that disruption of the redox process and stimulation of lipid peroxidation leads to cell damage and dysfunction of internal organs. This article presents recommendations for the prevention and treatment of these complications.
первичный миелофиброз, опухолевая интоксикация, спленомегалия, анемия, инфекционные осложнения, геморрагический синдром, экстрамедуллярное кроветворение, тромбоз, бластная трансформация, мочекислый диатез, вторичный гемосидерозprimary myelofibrosis, tumor intoxication, splenomegaly, anemia, infectious complications, hemorrhagic syndrome, extramedullary hematopoiesis, thrombosis, blast transformation, uric acid diathesis, secondarybirlamchi miyelofibroz, o'sma intoksikatsiyasi, splenomegaliya, anemiya, yuqumli asoratlar, gemorragik sindrom, ekstramedullar qon yaratish o'choqlari, tromboz, blast transformatsiyasi, siydik kislotasi diatezi, gemosideroz
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