MPLW515L Is a Novel Somatic Activating Mutation in Myelofibrosis with Myeloid Metaplasia

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Authors

Yana Pikman · Brigham and Women's Hospital

Benjamin H. Lee · Harvard University

Thomas Mercher · Brigham and Women's Hospital

Elizabeth McDowell · Harvard University

Benjamin L. Ebert · Broad Institute

Maricel Gozo · Brigham and Women's Hospital

Adam Cuker ·

Abstract

Activation of JAK-STAT signaling via MPLW515L is an important pathogenetic event in patients with JAK2V617F-negative MF. The bone marrow transplant model of MPLW515L-mediated myeloproliferative disorders (MPD) exhibits certain features of human MF, including extramedullary hematopoiesis, splenomegaly, and megakaryocytic proliferation. Further analysis of positive and negative regulators of the JAK-STAT pathway is warranted in JAK2V617F-negative MPD.

Topics & Keywords

Myeloproliferative Neoplasms: Diagnosis and Treatment Eosinophilic Disorders and Syndromes Kruppel-like factors research

UN Sustainable Development Goals

Good health and well-being

Publication Details

Published in: PLoS Medicine

Volume 3, Issue 7, pp. e270-e270

DOI: 10.1371/journal.pmed.0030270

Field-Weighted Citation Impact: 37.73