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爱泼斯坦-巴尔病毒相关弥漫性大B细胞淋巴瘤合并自身免疫性溶血性贫血和纯红细胞再生障碍性贫血。

Epstein-Barr virus associated diffuse large B-cell lymphoma complicated by autoimmune hemolytic anemia and pure red cell aplasia.

作者信息

Katayama H, Takeuchi M, Yoshino T, Munemasa M, Tada A, Soda R, Takahashi K

机构信息

Department of Internal Medicine, National Sanatrium Minami Okayama Hospital, 4066 Hayashima Cho, Tsukubo Gun, Okayama 701-0304, Japan.

出版信息

Leuk Lymphoma. 2001 Jul;42(3):539-42. doi: 10.3109/10428190109064614.

Abstract

A 53-year old man with systemic lymphadenopathy and hepatosplenomegaly was diagnosed with diffuse large B cell-lymphoma after inguinal lymph node biopsy. Anemia was noted, direct and indirect Coombs tests were positive, and the haptoglobin level was low. However, the bone marrow aspirate revealed erythroid aplasia. Co-existing autoimmune haemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were diagnosed. In situ hybridization with Epstein-Barr virus (EBV) encoded small RNA (EBER) showed positive findings in lymphoma cells. Southern blot hybridization revealed immunoglobulin heavy chain gene rearrangement and a clonal EBV terminal repeat, indicating monoclonal proliferation of EBV in infected B cells. The patient was treated with CHOP, resulting in a complete remission (CR). AIHA and PRCA subsided after 3 courses of chemotherapy. In conclusion, this case demonstrates not only the association of B-cell lymphoma with autoimmune disorders but also the involvement of EBV in these conditions.

摘要

一名53岁男性,伴有全身淋巴结肿大和肝脾肿大,经腹股沟淋巴结活检后被诊断为弥漫性大B细胞淋巴瘤。发现有贫血,直接和间接抗人球蛋白试验均为阳性,血清结合珠蛋白水平较低。然而,骨髓穿刺显示红系造血障碍。诊断为同时存在自身免疫性溶血性贫血(AIHA)和纯红细胞再生障碍性贫血(PRCA)。用爱泼斯坦-巴尔病毒(EBV)编码的小RNA(EBER)进行原位杂交,在淋巴瘤细胞中显示阳性结果。Southern印迹杂交显示免疫球蛋白重链基因重排和克隆性EBV末端重复序列,表明EBV在受感染的B细胞中呈单克隆增殖。该患者接受了CHOP方案治疗,实现了完全缓解(CR)。3个疗程的化疗后,AIHA和PRCA症状消退。总之,该病例不仅显示了B细胞淋巴瘤与自身免疫性疾病的关联,还表明了EBV在这些疾病中的作用。

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