Revista Brasileira de Hematologia e Hemoterapia ()

Primary myelofibrosis: current therapeutic options

  • Paula de Melo Campos

DOI
https://doi.org/10.1016/j.bjhh.2016.04.003
Journal volume & issue
Vol. 38, no. 3
pp. 257 – 263

Abstract

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ABSTRACT Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach.

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