Chronic myelomonocytic leukemia as a transformation from polycythemia vera

The hallmark of Polycythemia vera (PV) is the presence of JAK2V617F mutation and increased RBC mass. Chronic myelomonocytic leukemia (CMML) is defined as persistent blood absolute monocyte count (AMC) >/= 1 × 109/L for at least 3 months with myeloid cell dysplasia. Few cases of evolved CMML from...

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Bibliographic Details
Main Authors: Mirela Andrei, Anita Pandey, Boris Avezbakiyev, Jen Chin Wang
Format: Article
Language:English
Published: Elsevier 2019-01-01
Series:Leukemia Research Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2213048918300700
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Summary:The hallmark of Polycythemia vera (PV) is the presence of JAK2V617F mutation and increased RBC mass. Chronic myelomonocytic leukemia (CMML) is defined as persistent blood absolute monocyte count (AMC) >/= 1 × 109/L for at least 3 months with myeloid cell dysplasia. Few cases of evolved CMML from PV have been described. We present a case of PV that progressed to CMML. We demonstrated the CMML clone was most likely derived from PV- JAK2V617F clone. This clone carried a complex genetic mutations of ASXL1, RUNX1, SRSF2 and TET2, NRAS, KRAS, plus CMML cells were of the classical phenotype CD14+ CD16−by flow cytometry. Keywords: Chronic myelomonocytic leukemia, Polycythemia vera, Monocytosis
ISSN:2213-0489