TY - JOUR
T1 - SOHO State of the Art Updates and Next Questions
T2 - Myelofibrosis
AU - Pettit, Kristen
AU - Verstovsek, Srdan
AU - Talpaz, Moshe
PY - 2019/4/1
Y1 - 2019/4/1
N2 - The discovery of a mutation in the Janus Kinase 2 gene in 2005 spurred significant progress in the field of myeloproliferative neoplasms. A comprehensive description of genomic factors at play in the malignant clone in myeloproliferative neoplasms, particularly myelofibrosis (MF), have recently led to more precise, personalized prognostic tools. Despite this, understanding of the disease pathogenesis remains relatively limited. We continue to lack a detailed description of the interaction between the hematopoietic stem cell clone, abnormal bone marrow niche cells, and circulating signaling molecules and an understanding of how they cooperate to promote cell proliferation, fibrogenesis, and extramedullary hematopoiesis. Despite our knowledge gaps, recent research in MF has led to promising clinical translation. In this article, we summarize recent insights into MF pathophysiology, progress in the development of novel therapeutics, and opportunities for further advancement of the field.
AB - The discovery of a mutation in the Janus Kinase 2 gene in 2005 spurred significant progress in the field of myeloproliferative neoplasms. A comprehensive description of genomic factors at play in the malignant clone in myeloproliferative neoplasms, particularly myelofibrosis (MF), have recently led to more precise, personalized prognostic tools. Despite this, understanding of the disease pathogenesis remains relatively limited. We continue to lack a detailed description of the interaction between the hematopoietic stem cell clone, abnormal bone marrow niche cells, and circulating signaling molecules and an understanding of how they cooperate to promote cell proliferation, fibrogenesis, and extramedullary hematopoiesis. Despite our knowledge gaps, recent research in MF has led to promising clinical translation. In this article, we summarize recent insights into MF pathophysiology, progress in the development of novel therapeutics, and opportunities for further advancement of the field.
KW - Fibrosis
KW - Inflammation
KW - JAK inhibitors
KW - MPN
KW - Targeted therapy
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U2 - 10.1016/j.clml.2019.03.011
DO - 10.1016/j.clml.2019.03.011
M3 - Review article
C2 - 30987952
AN - SCOPUS:85064076699
SN - 2152-2650
VL - 19
SP - 191
EP - 199
JO - Clinical Lymphoma, Myeloma and Leukemia
JF - Clinical Lymphoma, Myeloma and Leukemia
IS - 4
ER -