Castleman病并发重型温冷双抗体型自身免疫性溶血性贫血及纯红细胞再生障碍性贫血危象1例并文献复习  

A case of Castleman's disease complicated with severe autoinmmne hemolytic anemia with both warm and cold autoantibodies and pure red cell anemia

作　　者：冯术青[1] 崔晓聪[1] 朱梦波[1] 闫兴双[1] 杜小梅[1] 李晓宇[1] 高峰[1] 

机构地区：[1]唐山钢铁集团有限责任公司医院血液肿瘤科,河北唐山063020

出　　处：《临床血液学杂志》2013年第6期783-785,共3页Journal of Clinical Hematology

摘　　要：目的:探讨Castleman病并发重型温冷双抗体型自身免疫性溶血性贫血(AIHA)及纯红细胞再生障碍性贫血(PRCA)危象的诊断及治疗措施。方法:回顾性分析1例Castleman病并发重型温冷双抗体型AIHA及PRCA危象患者的临床资料,并进行文献复习。结果:该患者以极重度贫血入院,伴有意识障碍,经过输注洗涤红细胞后意识转清。行血常规及血型检查时血液自凝,血型鉴定及交叉配血困难。血红蛋白最低为19.2g/L,网织红细胞计数为0.002,总胆红素及间接胆红素升高,Coombs实验阳性,Hams实验阴性,红细胞/粒细胞CD55、CD59抗原表达正常,免疫球蛋白IgG、IgM升高,补体C3下降,冷凝集素实验阳性,颈部淋巴结活检示Castleman病,骨髓细胞形态学检查提示PRCA。确诊为Castleman病并发温冷双抗体型AIHA及PRCA危象。经甲泼尼龙、环磷酰胺、环孢A等免疫抑制剂联合辅助血浆置换综合治疗措施,患者疾病在短期内得到有效控制。结论:Castleman病并发重型温冷双抗体型AIHA及PRCA危象临床少见,疾病诊断应强调进行详细的病史采集和对各种相关实验室检查的综合分析,以免误诊漏诊。多种免疫抑制剂的联合应用及积极的血浆置换等综合治疗手段的实施,能够提高治疗反应。Objective： To investigate the diagnosis and treatment of Castleman＇s disease （CD） complicated with severe autoinmmne hemolytic anemia （AIHA） with both warm and cold autoantibodies and pure red cell ane- mia （PRCA）. Method：We retrospectively analyzed the clinical data of a patient with CD complicated with severe AIHA with both warm and cold autoantibodies and PRCA,and reviewed the relevant literature. Result： The patient with extremely severe anemia on admission, accompanied with disturbance of consciousness. After transfusion con- sciousness cleared. The hematoglobin level and reticulocyte numbers were very low. Coombs test and cold aggluti- nin test were positive. Hams test was negative. The levels of IgG and IgM were high and complement C3 was low. The cervical lymphonode biopsy revealed CD and the bone marrow smear revealed PRCA. After treatment with multiple immunosuppressive agents such as methylprednisolone, cyclophosphamide and cyclosporine A combined with plasma exchange,the diseases were effectively controlled in the short term. Conclusion：CD complicated with severe AIHA with both warm and cold autoantibodies and PRCA is rare. So comprehensive analysis of history- talking and relevant laboratory examination should be made to diagnose the diseases and avoid misdiagnosis. Com- prehensive therapy with immunosuppressive agents and plasma exchange are effective.

关 键 词：CASTLEMAN病 贫血 溶血性  自身免疫性 温冷双抗体型 贫血  再生障碍性 纯红细胞 

分 类 号：R556.5[医药卫生—血液循环系统疾病]