Current treatment of myelofibrosis based on risk stratification of patients
Authors:
M. Palová; K. Indrák; A. Hluší; T. Szotkowski
Authors‘ workplace:
Hemato-onkologická klinika, LF UP a FN Olomouc
Published in:
Transfuze Hematol. dnes,19, 2013, No. 3, p. 163-170.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
Primary myelofibrosis belongs to the group of Ph1-negative myeloproliferative neoplasms, along with essential thrombocythemia and polycythemia vera, which may also progress to a fibrotic stage. Risk stratification of patients is especially important for proper therapeutic decision making and selection of high risk patients suitable for allogeneic stem cell transplantation, the only curative option for myelofibrosis. Transplantation carries a significant risk of mortality and morbidity, therefore it is recommended in patients with a median survival of less than 5 years. The recently introduced Dynamic International Prognostic Scoring System-plus prognostic model uses eight independent predictors of inferior survival. Janus 2 kinase inhibitors present a new targeted therapy affecting pathogenetic signal pathways. The results of on-going studies will show their long-term efficacy, safety and impact on overall survival.
Key words:
myelofibrosis, splenomegaly, JAK2V617F mutation, JAK2 inhibitors
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