获得性纯红细胞再生障碍67例临床分析  

Clinical analysis of 67 cases of pure red cell aplastic anemia

作　　者：王倩 马洁娴 李邦华 王小钦 胡琦[3] 张梦雪 Wang Qian;Ma Jiexian;Li Banghua;Wang Xiaoqin;Hu Qi;Zhang Mengxue(Department of Hematology,Huashan Hospital,Fudan University,Shanghai 200040,China)

机构地区：[1]复旦大学附属华山医院血液科,上海200040 [2]复旦大学附属华东医院血液科,上海200040 [3]上海市中医医院血液科,200071

出　　处：《中华血液学杂志》2020年第6期506-510,共5页Chinese Journal of Hematology

摘　　要：目的总结纯红细胞再生障碍(PRCA)的继发因素、实验室特点、治疗方案及预后。方法本研究为多中心回顾性临床研究,纳入2010年6月1日至2019年6月1日初诊的18岁以上PRCA患者进行随访,并根据不同治疗方案与药物减量速度等进行疗效、生存的比较分析。结果共纳入67例PRCA患者,其中继发性PRCA占44.8%(30/67),最常见的基础疾病为胸腺瘤(10例)和T细胞大颗粒淋巴细胞白血病(6例)。PRCA患者总缓解率(ORR)为85.7%,3年总体生存(OS)率为(74.3±7.5)%。单用环孢素A治疗缓解率稍高于单用激素或环孢素A联合糖皮质激素[90.0%(36/40)对75.0%(12/16),P=0.147]。环孢素A治疗达完全缓解或部分缓解3~6个月后,每次减量25 mg/d,减量间隔≥1个月组中位无病生存时间长于减量间隔<1个月组[未达到对15(95%CI 7~23)个月,P<0.001]。有62.5%(10/16)患者复发后按初始治疗方案或增加剂量再次治疗仍有效。结论PRCA可发生于多种疾病,缓解率与生存率高,单用环孢素A治疗效果好,起效且稳定后需缓慢减量以降低复发率。Objective To investigate secondary factors,laboratory features,treatment options,and prognosis of pure red cell aplastic anemia(PRCA).Methods This was a multicenter retrospective clinical study.Patients aged above 18 years newly diagnosed with PRCA between June 1,2010,and June 1,2019,were recruited as the main study object.A comparative analysis of remission rate and overall survival rate was made according to different treatment schemes adopted by patients and different drug reduction rates.Results A total of 67 patients with PRCA were included in this study and the secondary PRCA group accounted for 44.8%(30/67).The most common secondary factors were thymoma(n=10)and T-cell large lymphocytic leukemia(T-LGLL)(n=6).The overall response rate of PRCA was 85.7%and the 3-year overall survival rate of PRCA was(74.3±7.5)%.The remission rate of cyclosporine A alone was slightly higher than that of oral glucocorticoid alone or combined with glucocorticoid[90.0%(36/40)vs 75.0%(12/16),P=0.147].After patients applied with cyclosporine A treatment reached CR/PR and remained stable for 3-6 months,the dose of cyclosporine A was reduced by 25 mg each time.The cyclosporine A reduction interval of a 25 mg/d reduction in more than 1 month significantly prolonged the median disease-free survival compared with a 25 mg/d reduction in less than 1 month[not reached vs 15(95%CI 7-23)months,P<0.001].There were 62.5%(10/16)of patients who responded to the initial or incremental treatment regimen after relapse.Conclusion PRCA has features of various secondary factors,high overall survival rate,and high remission rate.Treatment with cyclosporine A alone is preferred,and cyclosporine A should be slowly tapered to reduce the risk of later relapse after it takes effect and patients reach a steady state.

关 键 词：纯红细胞再生障碍 环孢素A 药物减量 

分 类 号：R556.5[医药卫生—血液循环系统疾病]