【摘要】 目的 通过对一例肾移植术后并发纯红细胞再生障碍性贫血(pure red cell aplasia,PRCA)病例的回顾性分析,并结合文献系统复习PRCA的发病机制、临床表现、诊断及鉴别诊断、治疗原则,以期提高对本病的认识和重视。方法 46岁男性患者一例,肾移植术后2个月出现进行性血红蛋白下降,予促红细胞生成素(erythropoietin,EPO)补充、输血等治疗症状无改善,入我院行骨髓细胞学检查,最后诊断PRCA。根据以上临床资料,并结合相关文献系统复习接受实体器官移植的患者术后并发PRCA的临床特征。结果 患者经对症及药物治疗,贫血得以纠正,病情稳定。文献复习提示实体器官移植术后因免疫抑制剂的应用,容易并发感染,可能导致PRCA。PRCA的治疗以调整药物、大剂量静脉注射丙种球蛋白为主,一般预后良好。结论 接受实体器官移植的患者,一旦发生不明原因的血红蛋白进行性下降,排除出血因素后应立即怀疑PRCA可能,并及时进行网织红细胞计数和骨髓病理学检查;紧急状态下可选用新鲜洗涤的红细胞输血支持,治疗包括避免应用可能引起贫血的免疫抑制剂,调整EPO制剂类型等措施,并推荐大剂量丙种球蛋白的静脉应用(intravenous immunoglobulin,IVIG)辅助。更多还原

【Abstract】 Objective To report one case of pure red cell aplasia(PRCA) following renal transplantation,and to systematically review pathology,clinical manifestation,diagnosis and differential diagnosis and treatment principle of PRCA in order to improve the understanding of the disease.Methods A 46 years old male patient presented hemoglobin progressive decline after renal transplantation,and blood transfusion and EPO supplementation could not improve the symptom.The bone marrow examination revealed the diagnosis of PRCA.According to the above clinical data we summarized literatures.Results The patient got a stable state through the symptomatic treatment.Literature review showed that PRCA may be caused due to immunosuppression and intercurrent infection after the application of solid organ transplantation.After adjustment of drugs,and administration of IVIG,etc.,patients always obtain a good prognosis.Conclusions For patients undergoing solid organ transplantation who once presented unexplained hemoglobin decline,PRCA should be immediately suspected,and red blood cells are counted and bone marrow examination is carried out at the same time.The red blood cell transfusion support can be selected in emergency condition.Other treatment includes adjustment of the EPO preparation type,avoidance of application of immune inhibitor which can cause anemia,and intravenous immunoglobulin.更多还原