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Progress in elucidation of molecular pathophysiology of myeloproliferative neoplasms and its application to therapeutic decisions

  • Progress in Hematology
  • Progress in elucidation of molecular pathophysiology and its application to therapeutic decisions of MPNs
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Abstract

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-α are the established therapies in MF and high-risk PV patients.

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Acknowledgements

RK received the grant support by the Austrian Science Fund, FWF SFB F4702, P29018-B30.

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  1. Department of Laboratory Medicine, Medical University of Vienna, 18-20 Währinger Gürtel, 1090, Vienna, Austria

    Ruochen Jia & Robert Kralovics

  2. CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria

    Ruochen Jia & Robert Kralovics

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Jia, R., Kralovics, R. Progress in elucidation of molecular pathophysiology of myeloproliferative neoplasms and its application to therapeutic decisions. Int J Hematol 111, 182–191 (2020). https://doi.org/10.1007/s12185-019-02778-9

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