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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Main Authors: Xiaoyan Han, Chunxiang Jin, Gaofeng Zheng, Yi Li, Yungui Wang, Enfan Zhang, Honghu Zhu, Zhen Cai
Format: Article
Language:English
Published: SAGE Publishing 2021-01-01
Series:Therapeutic Advances in Hematology
Online Access:https://doi.org/10.1177/2040620720976984
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author Xiaoyan Han
Chunxiang Jin
Gaofeng Zheng
Yi Li
Yungui Wang
Enfan Zhang
Honghu Zhu
Zhen Cai
author_facet Xiaoyan Han
Chunxiang Jin
Gaofeng Zheng
Yi Li
Yungui Wang
Enfan Zhang
Honghu Zhu
Zhen Cai
author_sort Xiaoyan Han
collection DOAJ
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 doaj.art-911300e9a3454b0d887f4ad109c745132022-12-21T21:31:28ZengSAGE PublishingTherapeutic Advances in Hematology2040-62152021-01-011210.1177/2040620720976984Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltrationXiaoyan HanChunxiang JinGaofeng ZhengYi LiYungui WangEnfan ZhangHonghu ZhuZhen CaiSome 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.https://doi.org/10.1177/2040620720976984
spellingShingle Xiaoyan Han
Chunxiang Jin
Gaofeng Zheng
Yi Li
Yungui Wang
Enfan Zhang
Honghu Zhu
Zhen Cai
Acute myeloid leukemia with CPSF6–RARG fusion resembling acute promyelocytic leukemia with extramedullary infiltration
Therapeutic Advances in Hematology
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
url https://doi.org/10.1177/2040620720976984
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