获得性纯红细胞再生障碍性贫血35例临床分析  

Clinical analysis of 35 patients with acquired pure red cell aplasia

作　　者：井丽萍[1] 邵宗鸿[1] 刘鸿[1] 吴玉红[1] 施均[1] 白洁[1] 涂梅峰[1] 王化泉[1] 邢莉敏 杨崇礼[1] 

机构地区：[1]中国医学科学院中国协和医科大学血液学研究所血液病医院,天津300020

出　　处：《中国实用内科杂志》2005年第10期907-908,共2页Chinese Journal of Practical Internal Medicine

摘　　要：目的分析纯红细胞再生障碍性贫血(PRCA)患者的临床特点及发病机制.方法 1990-01～2003-04对中国医学科学院血液学研究所确诊为PRCA的35例患者,根据免疫学指标分为Ⅰ组(异常组)17例,Ⅱ组(正常组)18例.去除原发病后所有患者都给予联合药物治疗[雄激素、免疫抑制剂和(或)糖皮质激素].结果Ⅰ组中9例患者出现外周血T细胞亚群比例倒置,11例患者肿瘤坏死因子α升高,6例出现白细胞介素-2升高,治疗有效率70.6%,部分患者免疫学指标趋于正常.Ⅱ组治疗有效率86.7%.对复发病例原方案再次治疗有效.结论免疫异常是PRCA发病的主要机制,免疫抑制治疗多数有效,复发后再治疗仍有效.Objective To analyze the clinical features of pure red cell aplasia （ PRCA）, and to improve the recognition of its pathogenesis and treatment. Methods Among 35 PRCA patients from 1990 -01 to 2003 - 04 in our hospital, 17 patients （ group Ⅰ ） had immunologic abnormality, and the other 18 patients （ group Ⅱ ） were normal at every immunologic index. Removed the primary affection, all patients were given the combine treatment with drugs： androgen, immunosuppressive agent or/and glucocorticoid. Results Nine patients in group Ⅰ appeared T subgroup ratio inversion in peripheral blood, eleven patients had higher level than normal with TNFα, and six patients＇ IL- 2 level was higher. The response was 70. 6%. Some patients shifted to normal in immunologic index. The response was 86.7% in group Ⅱ. But 11 cases relapsed of the 25 cured and remission patients. They responded again to the initial therapy. Conclusion Dysimmunity is the most important pathogenesis in PRCA patients. Most patients respond to immunosuppression therapy. The relapse patients also respond to initial therapy.

关 键 词：贫血 再生障碍性 获得性免疫 

分 类 号：R5[医药卫生—内科学]