Wang Q, Ma J X, Li B H, Wang X Q, Hu Q, Zhang M X
Department of Hematology, Huashan Hospital, Fudan University, Shanghai 200040, China.
Department of Hematology, Huadong Hospital, Fudan University, Shanghai 200040, China.
Zhonghua Xue Ye Xue Za Zhi. 2020 Jun 14;41(6):506-510. doi: 10.3760/cma.j.issn.0253-2727.2020.06.013.
To investigate secondary factors, laboratory features, treatment options, and prognosis of pure red cell aplastic anemia (PRCA) . 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. 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 (=10) and T-cell large lymphocytic leukemia (T-LGLL) (=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) 75.0% (12/16) , =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 15 (95% 7-23) months, <0.001]. There were 62.5% (10/16) of patients who responded to the initial or incremental treatment regimen after relapse. 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.
为研究纯红细胞再生障碍性贫血（PRCA）的继发因素、实验室特征、治疗方案及预后。这是一项多中心回顾性临床研究。将2010年6月1日至2019年6月1日期间新诊断为PRCA的18岁以上患者作为主要研究对象。根据患者采用的不同治疗方案和不同的减药速率对缓解率和总生存率进行比较分析。本研究共纳入67例PRCA患者，继发性PRCA组占44.8%（30/67）。最常见的继发因素是胸腺瘤（=10）和T细胞大颗粒淋巴细胞白血病（T-LGLL）（=6）。PRCA的总缓解率为85.7%，3年总生存率为（74.3±7.5）%。单独使用环孢素A的缓解率略高于单独使用口服糖皮质激素或联合糖皮质激素的缓解率[90.0%（36/40）对75.0%（12/16），P=0.147]。应用环孢素A治疗的患者达到完全缓解/部分缓解并稳定3-6个月后，每次将环孢素A剂量减少25mg。与每月减少25mg/d且时间小于1个月相比，每月减少25mg/d且时间超过1个月的环孢素A减量间隔显著延长了无病生存期的中位数[未达到对15（95%CI 7-23）个月，P<0.001]。62.
