Overview of Myeloproliferative Neoplasms: History, Pathogenesis, Diagnostic Criteria, and Complications

Hematol Oncol Clin North Am. 2021 Apr;35(2):159-176. doi: 10.1016/j.hoc.2020.12.001. Epub 2021 Jan 26.

Abstract

Myeloproliferative disorders are a group of diseases morphologically linked by terminal myeloid cell expansion that frequently evolve from one clinical phenotype to another and eventually progress to acute myeloid leukemia. Diagnostic criteria for the Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) have been established by the World Health Organization and they are recognized as blood cancers. MPNs have a complex and incompletely understood pathogenesis that includes systemic inflammation, clonal hematopoiesis, and constitutive activation of the JAK-STAT pathway. Complications, such as thrombosis and progression to overt forms of myelofibrosis and acute leukemia, contribute significantly to morbidity and mortality of patients with MPN.

Keywords: Diagnostic criteria; Essential thrombocythemia; History; Myelofibrosis; Myeloproliferative neoplasms; Polycythemia vera.

Publication types

  • Review

MeSH terms

  • Humans
  • Myeloproliferative Disorders* / diagnosis
  • Myeloproliferative Disorders* / pathology
  • Neoplasms* / diagnosis
  • Neoplasms* / pathology