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
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author Andrei, Mirela
Pandey, Anita
Avezbakiyev, Boris
Wang, Jen Chin
author_facet Andrei, Mirela
Pandey, Anita
Avezbakiyev, Boris
Wang, Jen Chin
author_sort Andrei, Mirela
collection PubMed
description 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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spelling pubmed-64295372019-04-04 Chronic myelomonocytic leukemia as a transformation from polycythemia vera Andrei, Mirela Pandey, Anita Avezbakiyev, Boris Wang, Jen Chin Leuk Res Rep Article 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. Elsevier 2019-03-14 /pmc/articles/PMC6429537/ /pubmed/30949429 http://dx.doi.org/10.1016/j.lrr.2019.03.004 Text en © 2019 The Authors. Published by Elsevier Ltd. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Article
Andrei, Mirela
Pandey, Anita
Avezbakiyev, Boris
Wang, Jen Chin
Chronic myelomonocytic leukemia as a transformation from polycythemia vera
title Chronic myelomonocytic leukemia as a transformation from polycythemia vera
title_full Chronic myelomonocytic leukemia as a transformation from polycythemia vera
title_fullStr Chronic myelomonocytic leukemia as a transformation from polycythemia vera
title_full_unstemmed Chronic myelomonocytic leukemia as a transformation from polycythemia vera
title_short Chronic myelomonocytic leukemia as a transformation from polycythemia vera
title_sort chronic myelomonocytic leukemia as a transformation from polycythemia vera
topic Article
url 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
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