Journal
INTERNATIONAL JOURNAL OF HEMATOLOGY
Volume 111, Issue 2, Pages 182-191Publisher
SPRINGER JAPAN KK
DOI: 10.1007/s12185-019-02778-9
Keywords
Myeloproliferative neoplasms; Hematological malignancy; Janus kinase; Calreticulin; Thrombopoietin receptor
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Myeloproliferative neoplasms (MPNs) are hematological diseases that are driven by somatic mutations in hematopoietic stem and progenitor cells. These mutations include JAK2, CALR and MPL mutations as the main disease drivers, mutations driving clonal expansion, and mutations that contribute to progression of chronic MPNs to myelodysplasia and acute leukemia. JAK-STAT pathway has played a central role in the disease pathogenesis of MPNs. Mutant JAK2, CALR or MPL constitutively activates JAK-STAT pathway independent of the cytokine regulation. Symptomatic management is the primary goal of MPN therapy in ET and low-risk PV patients. JAK2 inhibitors and interferon-alpha are the established therapies in MF and high-risk PV patients.
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