A Novel NUP98/RARG Gene Fusion in Pediatric Acute Myeloid Leukemia Resembling Acute Promyelocytic Leukemia.

Jingying Zhang, Heping Shen, Hua Song, Diying Shen, Chan Liao, Meixin Fang, Yan Wang, Yongmin Tang, Honghu Zhu

Journal of pediatric hematology/oncology 2022 Apr 01

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Here, we introduced the first case of acute myeloid leukemia (AML) with RARG-NUP98 in a pediatric patient. The young male presented with structural and functional abnormalities similar to hypergranular acute promyelocytic leukemia, but was resistant to all transretinoic acids and arsenic trioxide. Till date, only 12 adult AML cases involving RARG rearrangement have been reported. At present, there is no standardized or optimal treatment option for this AML subtype. Disease management may typically require a joint treatment strategy involving chemotherapy, immunotherapy, and support therapy. In this study, we report the clinical manifestations and experimental results of a 10-year-old male and review other cases of RARG gene rearrangement reported in the literature. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.

Citation

Jingying Zhang, Heping Shen, Hua Song, Diying Shen, Chan Liao, Meixin Fang, Yan Wang, Yongmin Tang, Honghu Zhu. A Novel NUP98/RARG Gene Fusion in Pediatric Acute Myeloid Leukemia Resembling Acute Promyelocytic Leukemia. Journal of pediatric hematology/oncology. 2022 Apr 01;44(3):e665-e671