环孢菌素治疗大颗粒淋巴细胞白血病相关纯红细胞再生障碍研究  

Cyclosporine in the treatment of large granular lymphocytic leukemia associated pure red cell aplasia

作　　者：姜金龙[1] 刘晓庆 杨慧[2] 徐丹[1] 李瑞鑫 龙启强[3] 杨岩[4] 计成阜[1] 何广胜[2] 李建勇[2] JIANG Jinlong;LIU Xiao-qing;YANG Hui;XU Dan;LI Rui-xin;LONG Qi-qiang;YANG Yan;JI Cheng-fu;HE Guang-sheng;LI Jian-yong(Department of Hematology,Funing people's Hospital,Funing 224400,China;不详)

机构地区：[1]阜宁县人民医院血液科,江苏阜宁224400 [2]南京医科大学第一附属医院江苏省人民医院血液科,江苏南京210029 [3]南京市第二医院,南京中医药大学附属南京医院血液科,江苏南京210003 [4]吉林大学第一医院血液肿瘤中心,吉林长春130021

出　　处：《中国实用内科杂志》2021年第10期859-862,共4页Chinese Journal of Practical Internal Medicine

基　　金：江苏省医学重点项目(BL2014086);江苏省普通高校优势学科(JX10231801);国家中医药管理局行业专项(201407001-4)。

摘　　要：目的探讨环孢菌素(CsA)治疗成人大颗粒淋巴细胞白血病(LGLL)相关纯红细胞再生障碍(PRCA)患者的疗效,分析疗效相关因素。方法回顾性分析江苏省人民医院2009年9月至2020年8月间接受CsA治疗的44例成人LGLL相关PRCA患者的临床表现、实验室检查特征及治疗结果,分析疗效相关因素。结果44例LGLL相关PRCA患者接受CsA治疗;7例完全缓解、11例部分缓解、26例无效;总有效率为40.9%;完全缓解率为15.9%。疗效影响单因素及多因素分析示CsA疗效与STAT3基因突变无显著关联(P=0.691;P=0.688)。CsA减量或停药是复发最常见原因(80.0%);环磷酰胺(CTX)对复发LGLL相关PRCA有效率为60.0%。结论CsA治疗成人LGLL相关PRCA有一定疗效,不受STAT3基因突变阳性影响。最常见复发原因是CsA减量或停药,CTX对复发患者有一定疗效。Objective To assess response to cyclosporine(CsA)and related influence factors in patients with large granular lymphocytic leukemia(LGLL)associated pure red cell aplasia(PRCA).Methods The clinical manifestations and treatment outcomes of 44 adult LGLL-associated PRCA patients treated with CsA in Jiangsu Provincial People’s Hospital from September 2009 to August 2020 were retrospectively analyzed.Results 18 patients(40.9%)showed response to CsA;including 7 complete remission cases(15.9%)and 11 partial remission cases(25.0%).Univariate and multivariate analysis of CsA efficacy indicated that STAT3 gene mutation had no significant impaction on CsA response(P=0.691;P=0.688).Dosage reduction and discontinuation of CsA were the most common causes of recurrence(80%).Cyclophosphamide led to a response rate of 60%(6/10)in relapsed cohort.Conclusions The use of CsA in LGLLassociated PRCA patients is feasible;and is not significantly affected by STAT3 gene mutation.CsA reduction or discontinuation is closely related to recurrence.Cyclophosphamide shows favourable responses in relapsed cases.

关 键 词：纯红细胞再生障碍 大颗粒淋巴细胞白血病 环孢菌素 STAT3 突变 

分 类 号：R556.5[医药卫生—血液循环系统疾病]