获得性纯红细胞再生障碍67例临床分析

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.