Primary myelofibrosis: current therapeutic options

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Primary myelofibrosis: current therapeutic options

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 t...

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Background Primary myelofibrosis (PMF) is a myeloproliferative disorder resulting in progressive bone marrow fibrosis. In some PMF patients, a mutation in the gene coding for janus-associated kinase 2 (JAK2) results in cell proliferation and evasion of apoptosis. PMF typically occurs in older patients, and is associated with poor prognosis and low survival, with death occurring secondary to car...

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Myelofibrosis (MF) is a clonal stem cell disorder characterized by cytopenias, splenomegaly, marrow fibrosis, and systemic symptoms due to elevated inflammatory cytokines. MF is associated with decreased survival. The quality of life of patients with MF is similar to other advanced malignancies. Allogeneic hematopoietic cell transplantation is a curative treatment, but is applicable to a minori...

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ژورنال

عنوان ژورنال: Revista Brasileira de Hematologia e Hemoterapia

سال: 2016

ISSN: 1516-8484

DOI: 10.1016/j.bjhh.2016.04.003