重组人促红细胞生成素致纯红细胞再生障碍性贫血5例临床观察

目的观察重组人促红细胞生成素致纯红细胞再生障碍性贫血(PRCA)患者经激素/环孢素干预的临床疗效。方法回顾性分析2008年4月至2011年4月在吉林大学第二医院血液净化中心透析且明确诊断为PRCA的5例患者,给予激素/环孢素干预,并观察用药前后贫血纠正情况、用药情况及临床疗效。结果 5例患者中2例单独使用足量激素干预有效,2例无效患者将激素减至中等剂量并加用环孢素后贫血改善。此4例患者血红蛋白维持在70-90g/L不再继续上升,重新应用促红细胞生成素后血红蛋白恢复到正常水平。该4例患者在停用激素/环孢素后随访8个月以上未复发。另一例(病例5)患者口服激素45mg/日,应用3个月后无效,换用环孢素,贫血得到初步改善,未应用促红细胞生成素,血红蛋白维持在70-80g/L。结论足量激素对部分患者有效,部分无效患者加用环孢素能够有效改善贫血,在病情得到稳定控制后,可加用促红细胞生成素进一步纠正贫血。

Clinical observation on five cases of recombinant human erythropoietin induced pure red cell aplasia

Objective To observe the efficacy of five recombinant human erythropoietin induced pure red cell aplasia（PRCA） patients treated with hormone /cyclosporine.Methods From April,2008 to April,2011,five patients with PRCA were found in the hemodialysis center,Second Hospital of Jilin University.Their clinical data were collected and analysed,including hormonal/cyclosporine treatment,medication and clinical efficacy.Results Two of the five cases were effective with the adequate amount of hormone therapy alone,other two ineffective cases were improved after the addition of cyclosporine with the moderate doses hormone.After reapplying erythropoietin,those four patients＇ hemoglobin rose to the normal level.In the above following 8months,the four patients were not recrudescent.Another case（case 5） were treated with hormones 45mg/day,valid for three months after application.Then switched to cyclosporine,anemia has been improved.In the following days,the patient＇s hemoglobin maintained at 70-80g/L without erythropoietin.Conclusion Adequate amount of hormone was effective in part of the PRCA patients.Part of the void improved after the addition of cyclosporine.We can reapply erythropoietin to improve anemia in the stable period of the disease.