Primary myelofibrosis: own experience and news from diagnostic and treatment

• Main Authors: K. M. Abdulkadyrov, V. A. Shuvaev, I. S. Martynkevich
• Format: Article
• Language: Russian
• Published: ABV-press 2015-06-01
• Series: Onkogematologiâ
• Subjects: primary myelofibrosis; Janus kinase gene mutation; calreticulin gene mutation; prognostic scales; treatment algorithm; ruxolitinib
• Online Access: http://oncohematology.abvpress.ru/index.php/ongm/article/view/142

<p>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<br />recommended PMF treatment algorithm is listed. The treatment methods, target drugs (Janus kinases inhibitors) trials results are discussed.</p>