OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press14210.17650/1818-8346-2015-10-2-26-36HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CAREГЕМОБЛАСТОЗЫ: ДИАГНОСТИКА, ЛЕЧЕНИЕ, СОПРОВОДИТЕЛЬНАЯ ТЕРАПИЯPrimary myelofibrosis: own experience and news from diagnostic and treatmentПервичный миелофиброз: собственный опыт и новое в диагностике и леченииAbdulkadyrovK. M.АбдулкадыровК. М.
Russian Federation
ShuvaevV. A.ШуваевВ. А.
Russian Federation
shuvaev77@mail.ruMartynkevichI. S.МартынкевичИ. С.
Russian Federation
Russian Research Institute of Hematology and Transfusiology, Federal Medical and Biological AgencyФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии» Федерального медико-биологического агентства16, 2 nd Sovetskaya St., St. Petersburg, 191024, RussiaРоссия, 191024, Санкт-Петербург, ул. 2-я Советская, 160406201510226360406201504062015https://oncohematology.abvpress.ru/ongm/article/view/142

The newest advances in primary myelofibrosis (PMF) pathogenesis study, diagnostic and treatment approaches are presented in this article. The JAK-STAT signal pathway activation now recognized as main pathogenesis mechanism of PMF, it caused by JAK2, CALR, MPL genes mutations. Authors demonstrate their own data about epidemiology, clinical signs, diagnostic and treatment results of 315 PMF patients. The most frequent clinical symptoms are: anemia, leukocytosis, thrombocytosis, splenomegaly, constitutional symptoms. Diagnostic criteria, prognostic scales (including cytogenetic and molecular features) issues are reviewed. Intermediate-1 risk grade is in the most proportion of patients. Therecommended PMF treatment algorithm is listed. The treatment methods, target drugs (Janus kinases inhibitors) trials results are discussed.

В статье подробно освещены литературные данные о последних достижениях в изучении патогенеза, новейших разработках в диагностике и терапии первичного миелофиброза (ПМФ). Ведущим механизмом патогенеза ПМФ в настоящее время считается активация JAK-STAT сигнального пути, обусловленная мутациями генов JAK2, CALR, MPL и др. Авторами приведены материалы собственных наблюдений об эпидемиологии, клинических проявлениях, результатах обследования и лечения 315 больных ПМФ. Наиболее частыми клиническими симптомами на момент диагностики были: анемия, лейкоцитоз, тромбоцитоз, спленомегалия, симптомы опухолевой интоксикации. Представлены диагностические критерии, наиболее актуальные прогностические шкалы, включающие цитогенетические и молекулярно-генетические особенности заболевания. Наиболее часто в исследуемой группе определялся промежуточный-1 риск заболевания. Представлен рекомендуемый алгоритм терапии ПМФ. Изложены методы лечения, результаты использования таргетных препаратов (ингибиторов янускиназ).

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