Vol 8, No 4 (2017)
Review paper
Published online: 2018-02-24
New prognostic factors in primary myelofibrosis — a current state of knowledge
Hematologia 2017;8(4):271-279.
Abstract
Out of all Philadelphia-negative myeloproliferative neoplasms, primary myelofibrosis has the worst prognosis. Currently, an allogeneic hematopoietic stem cell transplantation (allo-HSCT) is currently the only curative method for patients with primary myelofibrosis. This procedure however bears a high risk of complications and is reserved for only those patients with short overall survival. The development of new drugs (ruxolitynib amongst others) was made possible thanks to an improved understanding of the pathogenesis of primary myelofibrosis. It was also observed that newly discovered genetical abnormalities constituted independent risk factors in patient with primary myelofibrosis. Furthermore, a few new proposals of risk prognostic scores have been developed. These risk scores are still not a gold standard and new studies are required to verify their use whenever risk groups have been better defined in primary myelofibrosis patients. In the new drug era, the main question is which patients should now receive allo-HSCT.
Keywords: primary myelofibrosisdriver mutationhigh risk mutationrisk factors
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