COMPLICATIONS OF PRIMARY MYELOFIBROSIS AND TACTICS OF THEIR TREATMEN
Keywords:
primary myelofibrosis, tumor intoxication, splenomegaly, anemia, infectious complications, hemorrhagic syndrome, extramedullary hematopoiesis, thrombosis, blast transformation, uric acid diathesis, secondaryAbstract
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.
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Статья поступила в редакцию 03.03.2024; одобрена после рецензирования 20.04.2024; принята к публикации 26.04.2024.
The article was submitted 03.03.2024; approved after reviewing 20.04.2024; accepted for publication 26.04.2024.
Информация об авторах:
Абдиев Каттабек Махматович-к.м.н.доцент. Самаркандский Государственный медицинский университет. Е-mail: sammi@sammi.uz, https://orcid.org/0000-0001-5468-9657
Источники финансирования: Работа не имела специального финансирования.
Конфликт интересов: Авторы декларируют отсутствие явных и потенциальных конфликтов интересов, связанных с публикацией настоящей статьи.
Вклад авторов:
Абдиев Каттабек Махматович — идеологическая концепция работы, редактирование статьи; сбор и анализ источников литературы, написание текста.
Information about the authors:
Kattabek Makhmutovich Abdiev-PhD, Associate Professor. Samarkand State Medical University. E-mail: sammi@sammi.uz , https://orcid.org/0000-0001-5468-9657
Sources of funding: The work did not receive any specific funding.
Conflict of interest: The authors declare no explicit or potential conflicts of interest associated with the publication of this article.
Contribution of the authors:
Kattabek Makhmutovich Abdiev — ideological concept of the work, editing the article; collection and analysis of literature sources, writing the text.
