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一名患有纯红细胞再生障碍性贫血和与胸腺瘤相关的自身免疫性溶血性贫血患者的红细胞生成中三种不同免疫紊乱的证明。

Demonstration of three distinct immunological disorders on erythropoiesis in a patient with pure red cell aplasia and autoimmune haemolytic anaemia associated with thymoma.

作者信息

Taniguchi S, Shibuya T, Morioka E, Okamura T, Okamura S, Inaba S, Niho Y

机构信息

First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.

出版信息

Br J Haematol. 1988 Apr;68(4):473-7. doi: 10.1111/j.1365-2141.1988.tb04239.x.

Abstract

A patient with pure red cell aplasia (PRCA) and autoimmune haemolytic anaemia (AIHA), associated with a thymoma which had already been removed, was studied in order to investigate the pathogenesis of PRCA and AIHA. The autoantibody eluted from the surface of the patient's red blood cells (RBC) reacted with the large E antigen of the Rh complex. Immunoglobulin-G (IgG) purified from the patient's serum suppressed CFU-E and BFU-E but not CFU-GM colony formation in the presence of complement. This antibody was not adsorbed with large E antigen. T-lymphocytes in the bone marrow suppressing autologous CFU-E and BFU-E colonies were demonstrated. Thus, three distinct immunological disorders on erythropoiesis were present in this patient with PRCA and AIHA associated with thymoma in a thymectomized state.

摘要

对一名患有纯红细胞再生障碍性贫血(PRCA)和自身免疫性溶血性贫血(AIHA)且伴有已切除胸腺瘤的患者进行了研究,以探讨PRCA和AIHA的发病机制。从患者红细胞(RBC)表面洗脱的自身抗体与Rh复合物的大E抗原发生反应。在补体存在的情况下,从患者血清中纯化的免疫球蛋白G(IgG)抑制CFU-E和BFU-E,但不抑制CFU-GM集落形成。该抗体不被大E抗原吸附。骨髓中抑制自体CFU-E和BFU-E集落的T淋巴细胞得到证实。因此,在这名处于胸腺切除状态且患有与胸腺瘤相关的PRCA和AIHA的患者中,存在三种不同的红细胞生成免疫紊乱。

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