【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。更多还原

【Abstract】 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.更多还原