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伴有B细胞分化早期阻滞并合并胎儿弯曲菌败血症的古德综合征。

Good's syndrome with a block in the early stage of B cell differentiation and complicated by Campylobacter fetus sepsis.

作者信息

Yamazaki K, Watanabe N, Hasegawa A, Yamaguchi E, Miyamoto K, Sakiyama Y, Kawakami Y

机构信息

First Department of Medicine, School of Medicine, Hokkaido University, Sapporo.

出版信息

Intern Med. 1994 Aug;33(8):496-500. doi: 10.2169/internalmedicine.33.496.

DOI:10.2169/internalmedicine.33.496
PMID:7803919
Abstract

A 63-year-old man was admitted for Campylobacter fetus sepsis and immunodeficiency syndrome with thymoma (Good's syndrome). Serological examination demonstrated hypoimmunoglobulinemia. Analysis of lymphocyte subsets in the peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. However, there were no abnormalities of cellular immunity. This is a rare case of Good's syndrome in Japan in which the pathogenic mechanism involved a block in the early stage of B cell differentiation. Moreover, this is the first case ever reported of Campylobacter fetus sepsis associated with Good's syndrome.

摘要

一名63岁男性因胎儿弯曲杆菌败血症及合并胸腺瘤的免疫缺陷综合征(古德综合征)入院。血清学检查显示免疫球蛋白低下。对外周血和骨髓中的淋巴细胞亚群分析表明,带有B细胞标志物的细胞比例显著降低。然而,细胞免疫并无异常。这是日本一例罕见的古德综合征病例,其致病机制涉及B细胞分化早期的阻滞。此外,这是首例报道的与古德综合征相关的胎儿弯曲杆菌败血症病例。

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