Journal
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
Volume 43, Issue -, Pages 78-81Publisher
WILEY
DOI: 10.1111/ijlh.13565
Keywords
BCR-ABL (Philadelphia)-negative myeloproliferative neoplasm; leukemic transformation; Myeloproliferative neoplasm blast phase (MPN-BP); TP53
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Philadelphia (BCR-ABL)-negative myeloproliferative neoplasms (MPNs) can transform into an accelerated or a blast phase with poor response to standard therapy. While MPN-BP show >= 20% blasts, they are not classified as AML and commonly harbor specific mutations.
Philadelphia (BCR-ABL)-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPN can transform into an accelerated or a blast phase, which is associated with poor response to standard therapy and low overall median survival. We present an interesting case of a patient with a history of PMF and progression and summarize the current studies on genetic features of myeloproliferative neoplasms in blast phase (MPN-BP) with an emphasis on PMF. Although MPN-BP show >= 20% blasts in peripheral blood or bone marrow, it is not considered as acute myeloid leukemia (AML) according to the WHO classification. While MPNs-BP typically lack genetic mutations seen in de novo AML, they commonly harbor IDH1/2, SRSF2, ASXL1, and TP53 mutations, similar to the genetic profiles of acute myeloid leukemia with myelodysplasia-related changes (AML-MRC).
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