期刊
FRONTIERS IN IMMUNOLOGY
卷 11, 期 -, 页码 -出版社
FRONTIERS MEDIA SA
DOI: 10.3389/fimmu.2020.02093
关键词
MPN; JAK2; CALR; MPL; inflammation; megakaryocytes; monocytes; mesenchymal stromal cells
类别
资金
- NIH [R01HL131835]
- MPN Research Foundation
- Gabrielle's Angel Foundation for Cancer Research
- German Research Foundation (DFG) [JU3104/2-1]
Philadelphia-negative myeloproliferative neoplasms (MPN) are malignant bone marrow (BM) disorders, typically arising from a single somatically mutated hematopoietic stem cell. The most commonly mutated genes,JAK2,CALR, andMPLlead to constitutively active JAK-STAT signaling. Common clinical features include myeloproliferation, splenomegaly and constitutional symptoms. This review covers the contributions of cellular components of MPN pathology (e.g., monocytes, megakaryocytes, and mesenchymal stromal cells) as well as cytokines and soluble mediators to the development of myelofibrosis (MF) and highlights recent therapeutic advances. These findings outline the importance of malignant and non-malignant BM constituents to the pathogenesis and treatment of MF.
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