Transformation of a chronic myeloproliferative neoplasm to acute myelogenous leukemia: Does anything work?

Madappa N. Kundranda, Raoul Tibes, Ruben A. Mesa

Research output: Contribution to journalArticle

30 Scopus citations

Abstract

The BCR/ABL-negative myeloproliferative neoplasms (MPNs) of essential thrombocythemia, polycythemia vera, and primary myelofibrosis, over the natural course of their disease, have an increasing predisposition to transform to overt acute myeloid leukemia (AML)-most appropriately referred to as MPN-blast phase (MPN-BP). Although this transformation is a rare event, once AML has occurred, it isassociated with a poor response to therapy and short survival. The molecular events leading to transformation are poorly defined. Currently, no therapy other than allogeneic stem cell transplantation (ASCT) has been demonstrated to alter the natural history of this disease. Multiple therapeutic investigations are currently ongoing, including early ASCT, hypomethylatingagents, and JAK2 inhibition, to try to alter the course of the disease and improve outcomes. This review focuses onthe latest advances in our understanding of the biology of leukemic transformation and current clinical therapies that are available for this patient population.

Original languageEnglish (US)
Pages (from-to)78-86
Number of pages9
JournalCurrent Hematologic Malignancy Reports
Volume7
Issue number1
DOIs
Publication statusPublished - Mar 1 2012

    Fingerprint

Keywords

  • AML
  • Acute myelogenous leukemia
  • Acute myeloid leukemia
  • Blast phase
  • Essential thrombocythemia
  • JAK2 inhibitors
  • Leukemic transformation
  • Myeloproliferative neoplasm
  • Pathogenesis
  • Polycythemia vera
  • Primary myelofibrosis
  • Treatment

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this