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一名患有纯红细胞再生障碍性贫血、重症肌无力和胸腺瘤患者的双系造血抑制与T淋巴细胞功能障碍

Bilineage hematopoietic inhibitor and T lymphocyte dysfunction in a patient with pure red cell aplasia, myasthenia gravis and thymoma.

作者信息

Murase T

机构信息

Department of Internal Medicine, Second Tokyo National Hospital, Japan.

出版信息

Exp Hematol. 1993 Mar;21(3):451-5.

PMID:8440342
Abstract

The case history, laboratory findings and clinical course of a patient with pure red cell aplasia (PRCA) combined with myasthenia gravis and thymoma are reported herein. In vitro study revealed bilineage complement-dependent IgG inhibitor(s) in both the granulocyte-macrophage and erythroblastic progenitor cells. His serum showed high anti-acetylcholine receptor antibody levels associated with activity of myasthenia gravis as well as PRCA. Patient history of thymectomy 7 years previously followed by extensive cutaneous candidiasis with abnormal T lymphocyte subsets (decreased T4/T8 ratio and increased number of activated T lymphocytes) in both the bone marrow and peripheral blood suggested primary T lymphocyte dysfunction, whereas the erythropoiesis was not inhibited by T lymphocytes. This case is of interest in the context of a possible immunological pathogenesis for other hematopoietic disorders, including some cases of aplastic anemia.

摘要

本文报告了一例纯红细胞再生障碍性贫血(PRCA)合并重症肌无力和胸腺瘤患者的病史、实验室检查结果及临床病程。体外研究显示,粒细胞-巨噬细胞和红系祖细胞中均存在双系补体依赖性IgG抑制剂。他的血清显示抗乙酰胆碱受体抗体水平升高,这与重症肌无力以及PRCA的活动有关。患者7年前接受胸腺切除术,随后出现广泛的皮肤念珠菌病,骨髓和外周血中T淋巴细胞亚群异常(T4/T8比值降低,活化T淋巴细胞数量增加),提示原发性T淋巴细胞功能障碍,而红细胞生成未受到T淋巴细胞的抑制。在其他造血系统疾病,包括一些再生障碍性贫血病例的可能免疫发病机制背景下,该病例具有一定意义。

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