Primary myelofibrosis: own experience and news from diagnostic and treatment
https://doi.org/10.17650/1818-8346-2015-10-2-26-36
Abstract
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. The
recommended PMF treatment algorithm is listed. The treatment methods, target drugs (Janus kinases inhibitors) trials results are discussed.
About the Authors
K. M. Abdulkadyrov
Russian Research Institute of Hematology and Transfusiology, Federal Medical and Biological Agency; 16, 2 nd Sovetskaya St., St. Petersburg, 191024, Russia
Russian Federation
Russian Federation
V. A. Shuvaev
Russian Research Institute of Hematology and Transfusiology, Federal Medical and Biological Agency; 16, 2 nd Sovetskaya St., St. Petersburg, 191024, Russia
Russian Federation
Russian Federation
I. S. Martynkevich
Russian Research Institute of Hematology and Transfusiology, Federal Medical and Biological Agency; 16, 2 nd Sovetskaya St., St. Petersburg, 191024, Russia
Russian Federation
Russian Federation
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Review
For citations:
Abdulkadyrov K.M., Shuvaev V.A., Martynkevich I.S. Primary myelofibrosis: own experience and news from diagnostic and treatment. Oncohematology. 2015;10(2):26-36. (In Russ.) https://doi.org/10.17650/1818-8346-2015-10-2-26-36
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