The Role of Megakaryocytes in Myelofibrosis

Hematol Oncol Clin North Am. 2021 Apr;35(2):191-203. doi: 10.1016/j.hoc.2020.11.004. Epub 2021 Jan 11.

Abstract

Megakaryocytes give rise to platelets, which have a wide variety of functions in coagulation, immune response, inflammation, and tissue repair. Dysregulation of megakaryocytes is a key feature of in the myeloproliferative neoplasms, especially myelofibrosis. Megakaryocytes are among the main drivers of myelofibrosis by promoting myeloproliferation and bone marrow fibrosis. In vivo targeting of megakaryocytes by genetic and pharmacologic approaches ameliorates the disease, underscoring the important role of megakaryocytes in myeloproliferative neoplasms. Here we review the current knowledge of the function of megakaryocytes in the JAK2, CALR, and MPL-mutant myeloproliferative neoplasms.

Keywords: JAK/STAT; Megakaryocyte; Myeloproliferative neoplasms.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Calreticulin / genetics
  • Humans
  • Janus Kinase 2 / genetics
  • Megakaryocytes*
  • Mutation
  • Myeloproliferative Disorders* / genetics
  • Neoplasms* / genetics
  • Primary Myelofibrosis* / genetics
  • Receptors, Thrombopoietin / genetics

Substances

  • CALR protein, human
  • Calreticulin
  • Receptors, Thrombopoietin
  • MPL protein, human
  • Janus Kinase 2