Myeloproliferative and lymphoproliferative disorders: State of the art

Elisa Rumi, Claudia Baratè, Giulia Benevolo, Margherita Maffioli, Alessandra Ricco, Emanuela Sant'Antonio

Research output: Contribution to journalReview article

Abstract

Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are clonal disorders complicated mainly by vascular events and transformation to myelofibrosis (for PV and ET) or leukemia. Although secondary malignancies, in particular, lymphoproliferative disorders (LPNs), are rare, they occur at a higher frequency than found in the general population, and there has been recent scientific discussion regarding a hypothetical relationship between treatment with JAK inhibitors in MPN and the risk of development of LPN. This has prompted increased interest regarding the coexistence of MPN and LPN. This review focuses on the role of JAK2 and the JAK/STAT pathway in MPN and LPN, whether there is a role for the genetic background in the occurrence of both MPN and LPN and whether there is a role for cytoreductive drugs in the occurrence of both MPN and LPN. Furthermore, whether an increased risk of lymphoma development is limited to patients who receive the JAK inhibitor ruxolitinib, is a more general phenomenon that occurs following JAK1/2 inhibition or is associated with preferential JAK1 or JAK2 targeting is discussed.

Original languageEnglish
Pages (from-to)121-128
Number of pages8
JournalHematological Oncology
Volume38
Issue number2
DOIs
Publication statusPublished - Apr 1 2020

Keywords

  • JAK inhibitors
  • JAK/STAT pathway
  • JAK2
  • lymphoproliferative disorders
  • myeloproliferative neoplasms
  • ruxolitinib

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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