Advances in key pathogenesis of atypical acute promyelocytic leukemia and mechanisms of all-trans retinoic acid resistance

VernacularTitle:不典型急性早幼粒细胞白血病关键发病和全反式维甲酸耐药机制研究进展
Author: Xiaosu ZHOU ¹ ; Jiaqi CHEN ; Xue CHEN ; Hongxing LIU
Author Information

1. 北京陆道培血液病研究院精准医学中心，北京　100176
Keywords: Acute promyelocytic leukemia; Fusion gene; All-trans retinoic acid; Drug resistance
From: Journal of Leukemia & Lymphoma 2024;33(2):82-85
CountryChina
Language:Chinese
Abstract: Due to the successful application of all-trans retinoic acid (ATRA) and arsenic, the treatment of acute promyelocytic leukemia (APL) with PML::RARA fusion gene has achieved great success. However, some patients are presented with APL phenotype in cellular morphology, immunophenotype, and gene expression profile, while PML::RARA is negative, which is known as atypical APL (aAPL). In aAPL patients, more than 20 fusion genes related to retinoic acid receptors have been reported. It has been discovered that all evaluable patients with RARG fusion genes and approximately half of those with rare RARA fusion genes are resistant to ATRA, however, the molecular mechanisms of this resistance remain poorly studied. Combining with the reports in the 65th American Society of Hematology Annual Meeting, this paper reports great progresses of the key pathogenesis of aAPL and ATRA resistance mechanisms.