Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia

Retinoic acid receptor gamma (RARG) gene rearrangement has been reported in several acute myeloid leukemia (AML) patients. They resemble classical acute promyelocytic leukemia (APL) patients in clinical features, morphology, and immunophenotype but do not carry the promyelocytic leukemia (PML)–RARA...

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Autores principales: Zhao, Junmei, Wang, Wentao, Yan, Li, Chen, Xi, Li, Wen, Li, Wanying, Chen, Tingting, Chen, Lunhua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Oncology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9523789/
https://www.ncbi.nlm.nih.gov/pubmed/36185228
http://dx.doi.org/10.3389/fonc.2022.1011023
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author Zhao, Junmei
Wang, Wentao
Yan, Li
Chen, Xi
Li, Wen
Li, Wanying
Chen, Tingting
Chen, Lunhua
author_facet Zhao, Junmei
Wang, Wentao
Yan, Li
Chen, Xi
Li, Wen
Li, Wanying
Chen, Tingting
Chen, Lunhua
author_sort Zhao, Junmei
collection PubMed
description Retinoic acid receptor gamma (RARG) gene rearrangement has been reported in several acute myeloid leukemia (AML) patients. They resemble classical acute promyelocytic leukemia (APL) patients in clinical features, morphology, and immunophenotype but do not carry the promyelocytic leukemia (PML)–RARA fusion gene. Importantly, almost all these APL-like AML patients show resistance to all-trans retinoic acid (ATRA), and no effective treatment is recommended for them. Here, we identified a case of AML resembling APL in clinical presentation and experimental findings carrying a rare cleavage and polyadenylation-specific factor 6 (CPSF6)-RARG fusion gene. The patient was insensitive to ATRA and ATO but responded well to homoharringtonine and cytarabine.
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spelling pubmed-95237892022-10-01 Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia Zhao, Junmei Wang, Wentao Yan, Li Chen, Xi Li, Wen Li, Wanying Chen, Tingting Chen, Lunhua Front Oncol Oncology Retinoic acid receptor gamma (RARG) gene rearrangement has been reported in several acute myeloid leukemia (AML) patients. They resemble classical acute promyelocytic leukemia (APL) patients in clinical features, morphology, and immunophenotype but do not carry the promyelocytic leukemia (PML)–RARA fusion gene. Importantly, almost all these APL-like AML patients show resistance to all-trans retinoic acid (ATRA), and no effective treatment is recommended for them. Here, we identified a case of AML resembling APL in clinical presentation and experimental findings carrying a rare cleavage and polyadenylation-specific factor 6 (CPSF6)-RARG fusion gene. The patient was insensitive to ATRA and ATO but responded well to homoharringtonine and cytarabine. Frontiers Media S.A. 2022-09-15 /pmc/articles/PMC9523789/ /pubmed/36185228 http://dx.doi.org/10.3389/fonc.2022.1011023 Text en Copyright © 2022 Zhao, Wang, Yan, Chen, Li, Li, Chen and Chen https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Zhao, Junmei
Wang, Wentao
Yan, Li
Chen, Xi
Li, Wen
Li, Wanying
Chen, Tingting
Chen, Lunhua
Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia
title Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia
title_full Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia
title_fullStr Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia
title_full_unstemmed Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia
title_short Case report: A rare case of acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia
title_sort case report: a rare case of acute myeloid leukemia with cpsf6–rarg fusion resembling acute promyelocytic leukemia
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9523789/
https://www.ncbi.nlm.nih.gov/pubmed/36185228
http://dx.doi.org/10.3389/fonc.2022.1011023
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