Chronic myelomonocytic leukemia as a transformation from polycythemia vera

The hallmark of Polycythemia vera (PV) is the presence of JAK2(V617F) mutation and increased RBC mass. Chronic myelomonocytic leukemia (CMML) is defined as persistent blood absolute monocyte count (AMC) >/= 1 × 10(9)/L for at least 3 months with myeloid cell dysplasia. Few cases of evolved CMML f...

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Detalles Bibliográficos
Autores principales: Andrei, Mirela, Pandey, Anita, Avezbakiyev, Boris, Wang, Jen Chin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6429537/
https://www.ncbi.nlm.nih.gov/pubmed/30949429
http://dx.doi.org/10.1016/j.lrr.2019.03.004
Descripción
Sumario:The hallmark of Polycythemia vera (PV) is the presence of JAK2(V617F) mutation and increased RBC mass. Chronic myelomonocytic leukemia (CMML) is defined as persistent blood absolute monocyte count (AMC) >/= 1 × 10(9)/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- JAK2(V617F) 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.

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