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
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