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1例与自身免疫性溶血性贫血相关的γ重链病:临床、血液学、免疫学及病理学详情

A case of gamma heavy chain disease associated with autoimmune haemolytic anaemia: clinical, haematological, immunological and pathological details.

作者信息

Shirakura T, Kobayashi Y, Murai Y, Inoue T, Imamura Y

出版信息

Scand J Haematol. 1976 May;16(5):387-93. doi: 10.1111/j.1600-0609.1976.tb00332.x.

DOI:10.1111/j.1600-0609.1976.tb00332.x
PMID:821137
Abstract

A 76-year-old Japanese man, presented with a case of gamma heavy chain disease associated with autoimmune haemolytic anaemia. The clinical course progressed rapidly, accompanied by fever, lymphadenopathy, erythematous palate and hepatosplenomegaly. As the disease progressed, haemoglobin level gradually reduced, and jaundice occurred. A complication of autoimmune haemolytic anaemia was deduced from the laboratory data obtained at this time. The patient's serum and urine contained an M-component with electrophoretic and antigenic properties resembling those of the Fc-fragment of G1 globulin. Microscopic and electron microscopic findings on the tumor cells infiltrating in lymphnodes indicated all types of transitional forms from small lymphocytes to large lymphoplasmoblastic cells.

摘要

一名76岁的日本男性,被诊断为与自身免疫性溶血性贫血相关的γ重链病。临床病程进展迅速,伴有发热、淋巴结病、腭部红斑和肝脾肿大。随着病情进展,血红蛋白水平逐渐降低,并出现黄疸。根据此时获得的实验室数据推断为自身免疫性溶血性贫血并发症。患者的血清和尿液中含有一种M成分,其电泳和抗原特性类似于G1球蛋白的Fc片段。对浸润在淋巴结中的肿瘤细胞进行显微镜和电子显微镜检查发现,存在从小淋巴细胞到大型淋巴浆母细胞的各种过渡形式。

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