伴STAT5b::RARα融合基因急性早幼粒细胞白血病1例并文献复习  

Acute promyelocytic leukemia with STAT5b::RARαfusion gene:report of 1 case and review of literature

作　　者：郭强[1] 樊红琼[1] 高素君[1] 杜忠华[1] 白晶[1] 刘秋菊[1] Guo Qiang;Fan Hongqiong;Gao Sujun;Du Zhonghua;Bai Jing;Liu Qiuju(Department of Hematology,the First Hospital of Jilin University,Changchun 130061,China)

机构地区：[1]吉林大学第一医院血液科,长春130061

出　　处：《白血病．淋巴瘤》2024年第4期224-227,共4页Journal of Leukemia & Lymphoma

摘　　要：目的探讨伴STAT5b::RARα融合基因的急性早幼粒细胞白血病(APL)患者的临床特征及预后。方法回顾性分析2023年5月吉林大学第一医院收治的1例以及国内外相关文献报道的18例伴STAT5b::RARα融合基因的APL患者的临床资料,对其临床特征、临床疗效进行总结。结果19例患者中,男性15例,女性4例;年龄[M(Q1,Q3)]32岁(27岁,47岁),APL高危组6例,低危组13例;15例表达CD13、CD33,3例表达CD34,4例表达HLA-DR,11例表达CD117。14例伴随附加染色体异常。全反式维A酸联合化疗一线缓解率26%(5/19),二线诱导缓解率25%(3/12),至截稿前6例生存。结论伴STAT5b::RARα融合基因APL患者骨髓形态及免疫表型与经典APL相仿,但更易出现附加染色体异常,治疗缓解率低,总体预后差。Objective To investigate the clinical features and prognosis of acute promyelocytic leukemia(APL)patients with STAT5b::RARαfusion gene.Methods The clinical data of 1 APL patient with STAT5b::RARαfusion gene in the First Hospital of Jilin University on May 2023,as well as 18 APL patients with STAT5b::RARαfusion gene reported in relevant domestic and international literature,were retrospectively analyzed,and their clinical characteristics and clinical efficacy were summarized.Results Among 19 patients,15 were male and 4 were female,and the age[M(Q1,Q3)]was 32 years old(27 years old,47 years old).There were 6 cases in the high-risk group and 13 cases in the low-risk group.Fifteen patients expressed CD13 and CD33,3 cases expressed CD34,4 cases expressed HLA-DR,and 11 cases expressed CD117.Fourteen patients had additional chromosomal abnormalities.The first-line remission rate of all-trans retinoic combined with chemotherapy was 26%(5/19),and the second-line induction remission rate was 25%(3/12),and 6 patients were alive before the deadline.Conclusions The bone marrow morphology and immunophenotype of APL patients with STAT5b::RARαfusion gene are similar to classical APL,but additional chromosomal abnormalities are more likely to occur;the treatment remission rate is low,and the overall prognosis is poor.

关 键 词：白血病 早幼粒细胞 急性 STAT5b::RARα 治疗结果 预后 

分 类 号：R733.71[医药卫生—肿瘤]