Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration

Some subtypes of acute myeloid leukemia (AML) share morphologic, immunophenotypic, and clinical features of acute promyelocytic leukemia (APL), but lack a PML–RARA (promyelocytic leukemia–retinoic acid receptor alpha) fusion gene. Instead, they have the retinoic acid receptor beta (RARB) or retinoic...

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Autores principales: Han, Xiaoyan, Jin, Chunxiang, Zheng, Gaofeng, Li, Yi, Wang, Yungui, Zhang, Enfan, Zhu, Honghu, Cai, Zhen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7797573/
https://www.ncbi.nlm.nih.gov/pubmed/33473264
http://dx.doi.org/10.1177/2040620720976984
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author Han, Xiaoyan
Jin, Chunxiang
Zheng, Gaofeng
Li, Yi
Wang, Yungui
Zhang, Enfan
Zhu, Honghu
Cai, Zhen
author_facet Han, Xiaoyan
Jin, Chunxiang
Zheng, Gaofeng
Li, Yi
Wang, Yungui
Zhang, Enfan
Zhu, Honghu
Cai, Zhen
author_sort Han, Xiaoyan
collection PubMed
description Some subtypes of acute myeloid leukemia (AML) share morphologic, immunophenotypic, and clinical features of acute promyelocytic leukemia (APL), but lack a PML–RARA (promyelocytic leukemia–retinoic acid receptor alpha) fusion gene. Instead, they have the retinoic acid receptor beta (RARB) or retinoic acid receptor gamma (RARG) rearranged. Almost all of these AML subtypes exhibit resistance to all-trans retinoic acid (ATRA); undoubtedly, the prognosis is poor. Here, we present an AML patient resembling APL with a novel cleavage and polyadenylation specific factor 6 (CPSF6)–RARG fusion, showing resistance to ATRA and poor response to chemotherapy with homoharringtonine and cytarabine. Simultaneously, the patient also had extramedullary infiltration.
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spelling pubmed-77975732021-01-19 Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration Han, Xiaoyan Jin, Chunxiang Zheng, Gaofeng Li, Yi Wang, Yungui Zhang, Enfan Zhu, Honghu Cai, Zhen Ther Adv Hematol Case Report Some subtypes of acute myeloid leukemia (AML) share morphologic, immunophenotypic, and clinical features of acute promyelocytic leukemia (APL), but lack a PML–RARA (promyelocytic leukemia–retinoic acid receptor alpha) fusion gene. Instead, they have the retinoic acid receptor beta (RARB) or retinoic acid receptor gamma (RARG) rearranged. Almost all of these AML subtypes exhibit resistance to all-trans retinoic acid (ATRA); undoubtedly, the prognosis is poor. Here, we present an AML patient resembling APL with a novel cleavage and polyadenylation specific factor 6 (CPSF6)–RARG fusion, showing resistance to ATRA and poor response to chemotherapy with homoharringtonine and cytarabine. Simultaneously, the patient also had extramedullary infiltration. SAGE Publications 2021-01-09 /pmc/articles/PMC7797573/ /pubmed/33473264 http://dx.doi.org/10.1177/2040620720976984 Text en © The Author(s), 2020 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Han, Xiaoyan
Jin, Chunxiang
Zheng, Gaofeng
Li, Yi
Wang, Yungui
Zhang, Enfan
Zhu, Honghu
Cai, Zhen
Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
title Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
title_full Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
title_fullStr Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
title_full_unstemmed Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
title_short Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
title_sort acute myeloid leukemia with cpsf6–rarg fusion resembling acute promyelocytic leukemia with extramedullary infiltration
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7797573/
https://www.ncbi.nlm.nih.gov/pubmed/33473264
http://dx.doi.org/10.1177/2040620720976984
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