Current Hematologic Malignancy Reports

, Volume 7, Issue 1, pp 78–86

Transformation of a Chronic Myeloproliferative Neoplasm to Acute Myelogenous Leukemia: Does Anything Work?

  • Madappa N. Kundranda
  • Raoul Tibes
  • Ruben A. Mesa
Acute Myelogenous Leukemia (EJ Feldman, Section Editor)

DOI: 10.1007/s11899-011-0107-9

Cite this article as:
Kundranda, M.N., Tibes, R. & Mesa, R.A. Curr Hematol Malig Rep (2012) 7: 78. doi:10.1007/s11899-011-0107-9

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 is associated 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, hypomethylating agents, and JAK2 inhibition, to try to alter the course of the disease and improve outcomes. This review focuses on the latest advances in our understanding of the biology of leukemic transformation and current clinical therapies that are available for this patient population.

Keywords

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

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Madappa N. Kundranda
    • 1
  • Raoul Tibes
    • 1
  • Ruben A. Mesa
    • 2
  1. 1.Division of Hematology/Oncology, Department of Internal MedicineMayo ClinicScottsdaleUSA
  2. 2.Consultant of Hematology & OncologyMayo ClinicScottsdaleUSA

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