呈现急性早幼粒细胞白血病表现的特殊亚型白血病  

Special subtypes of leukemia presenting with acute promyelocytic leukemia

作　　者：金春香 韩晓雁[1] 蔡真[1] JIN Chunxiang;HAN Xiaoyan;CAI Zhen

机构地区：[1]浙江大学附属第一医院骨髓移植中心,杭州310003

出　　处：《临床血液学杂志》2020年第6期811-816,共6页Journal of Clinical Hematology

摘　　要：急性早幼粒细胞白血病(acute promyelocytic leukemia,APL)是急性髓系白血病(acute myeloid leukemia,AML)的一种特殊亚型,占其10%~15%[1],含嗜天青颗粒的早幼粒细胞在骨髓和外周血中异常增殖是其独特的形态学特征。APL具有严重的出血倾向,并可快速进展至弥散性血管内凝血,曾被认为是AML中恶性程度最高的类型[2]。t(15;17)染色体易位形成PML-RARA融合基因,被发现是维甲酸(ATRA)诱导分化治疗的靶点,同时采用ATRA和蒽环类化疗药物的标准诱导治疗方案可实现90%~95%的完全缓解率(CR)[3],但许多患者出现了延迟复发。砷剂(ATO)联合ATRA的一线应用从根本上改变了这类疾病的预后,即使不进行化疗也完全可能治愈,APL已成为AML中预后最好的亚型。Some subtypes of acute myeloid leukemia(AML)share morphologic,immunophenotypic and clinical features of acute promyelocytic leukemia(APL),but absent PML-RARA fusion genes.In some cases,RARA was fuse with other partner genes,the sensitivity of these APL variants to all-trans retinoic acid(ATRA)was different.Furthermore,a few of AML patients resembling typical APL had the retinoic acid receptor beta(RARB)or retinoic acid receptor gamma(RARG)rearranged,and most of these subtypes of AML exhibited resistance to ATRA,with undoubtedly poor the prognosis.In this review,we will discuss these special subtypes of leukemia,in order to early diagnose and improve the prognosis of these patients.

关 键 词：变异型急性早幼粒细胞白血病 RARB重排 RARG重排 维甲酸耐药 

分 类 号：R733.71[医药卫生—肿瘤]