Clinical analysis of 100 adult patients with pure red cell aplasia.
- Author:
Hua YIN
1
;
Xiao-qing LIU
1
;
Lang CHENG
1
;
Zheng-yuan LIU
1
;
Rui-nan LU
1
;
Ming HONG
1
;
Yu ZHU
1
;
Hua-yuan ZHU
1
;
Jian-fu ZHANG
1
;
Guang-sheng HE
1
;
Jian-yong LI
1
Author Information
- Publication Type:Journal Article
- Keywords: pure red cell aplasia; idiopathic; secondary; large granular lymphocytic leukemia; cyclospoine A
- From: Chinese Journal of Practical Internal Medicine 2019;39(10):891-895
- CountryChina
- Language:Chinese
- Abstract: OBJECTIVE: To evaluate clinical characteristics and treatment response of 100 patients with pure red cell aplasia(PRCA).METHODS: We retrospectively analyzed the clinical data of 100 adult patients with acquired PRCA from October2009 to July 2019, and compared the difference in efficacy between idiopathic and secondary patients.RESULTS: 100 patients were evaluated, including 60 idiopathic patients and 40 secondary patients.The most common reasons for secondary PRCA were large granular lymphocytic leukemia(LGLL)(28 cases,70.0%)and thymoma(6 cases, 15.0%). The remission induced regimens included corticosteroids(CS), cyclosporine A(CsA), or other agents, and the response rate were 66.7%,71.4% and 50%, respectively(P=0.336). Secondary PRCA was less effective than idiopathic PRCA(52.5%,78.3%,P=0.007). PRCA related to large granular lymphocytic leukemia was also less effective compared to idiopathic PRCA(46.4%,79.3%,P=0.003). When treated by CsA, idiopathic PRCA was more effective than secondary PRCA and LGLL related PRCA(P=0.001, P=0.000). Logistic regression analysis showed that lower response rate was related to secondary PRCA and LGLL related PRCA.CONCLUSION: The response rate were similar by different induced regimens. Idiopathic PRCA could acquired better response to CsA than secondary, LGLL related PRCA was less effective to treatment.
