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Association of Myelofibrosis Phenotypes with Clinical Manifestations, Molecular Profiles, and Treatments.


ABSTRACT: Myelofibrosis (MF) presents an array of clinical manifestations and molecular profiles. The two distinct phenotypes- myeloproliferative and myelodepletive or cytopenic- are situated at the two poles of the disease spectrum and are largely defined by different degrees of cytopenias, splenomegaly, and distinct molecular profiles. The myeloproliferative phenotype is characterized by normal/higher peripheral blood counts or mildly decreased hemoglobin, progressive splenomegaly, and constitutional symptoms. The myeloproliferative phenotype is typically associated with secondary MF, higher JAK2 V617F burden, fewer mutations, and superior overall survival (OS). The myelodepletive phenotype is usually associated with primary MF, ≥2 cytopenias, modest splenomegaly, lower JAK2 V617F bu

SUBMITTER: Chifotides HT 

PROVIDER: S-EPMC10340291 | biostudies-literature | 2023 Jun

REPOSITORIES: biostudies-literature