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