Clinical analysis of 67 cases of pure red cell aplastic anemia
10.3760/cma.j.issn.0253-2727.2020.06.013
- VernacularTitle:获得性纯红细胞再生障碍67例临床分析
- Author:
Qian WANG
1
;
Jiexian MA
;
Banghua LI
;
Xiaoqin WANG
;
Qi HU
;
Mengxue ZHANG
Author Information
1. 复旦大学附属华山医院血液科，上海 200040
- Keywords:
Pure red cell aplastic anemia;
Cyclosporine A;
Dosage reduction
- From:
Chinese Journal of Hematology
2020;41(6):506-510
- CountryChina
- Language:Chinese
-
Abstract:
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.
