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

  • Acute Myelogenous Leukemia (EJ Feldman, Section Editor)
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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.

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Authors and Affiliations

  1. Division of Hematology/Oncology, Department of Internal Medicine, Mayo Clinic, Scottsdale, AZ, USA

    Madappa N. Kundranda & Raoul Tibes

  2. Consultant of Hematology & Oncology, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ, 85259, USA

    Ruben A. Mesa

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  1. Madappa N. Kundranda
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  2. Raoul Tibes
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  3. Ruben A. Mesa
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Correspondence to Ruben A. Mesa.

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Kundranda, M.N., Tibes, R. & Mesa, R.A. Transformation of a Chronic Myeloproliferative Neoplasm to Acute Myelogenous Leukemia: Does Anything Work?. Curr Hematol Malig Rep 7, 78–86 (2012). https://doi.org/10.1007/s11899-011-0107-9

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