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与胸腺瘤相关的持续性低丙种球蛋白血症(古德综合征)。

Durable hypogammaglobulinemia associated with thymoma (Good syndrome).

作者信息

Kitamura Atsushi, Takiguchi Yuichi, Tochigi Naobumi, Watanabe Shun-ichi, Sakao Seiichirou, Kurosu Katsushi, Tanabe Nobuhiro, Tatsumi Koichiro

机构信息

Department of Respirology, Graduate School of Medicine, Chiba University, Chiba.

出版信息

Intern Med. 2009;48(19):1749-52. doi: 10.2169/internalmedicine.48.2375. Epub 2009 Oct 1.

Abstract

Good syndrome, characterized by hypogammaglobulinemia and acquired immunodeficiency, is a rare condition associated with thymoma. A 67-year-old woman, who 4 months previously had a thymoma resected, presented with generalized hypogammaglobulinemia with a severely decreased B cell population as demonstrated by flow cytometry. She was diagnosed as having bacterial mediastinitis associated with Good syndrome. For the subsequent 6 years, she suffered from repeated serious bacterial infections. As this paraneoplastic syndrome is not resolved by tumor removal, careful management with intensive infection-control using antibiotics and intravenous immunoglobulins is required for the long term. Serum immunoglobulin levels should be evaluated for patients with thymoma and suspected vulnerability to infection.

摘要

古德综合征以低丙种球蛋白血症和获得性免疫缺陷为特征,是一种与胸腺瘤相关的罕见病症。一名67岁女性,4个月前接受了胸腺瘤切除术,现出现全身性低丙种球蛋白血症,流式细胞术显示B细胞数量严重减少。她被诊断为与古德综合征相关的细菌性纵隔炎。在随后的6年里,她反复遭受严重的细菌感染。由于这种副肿瘤综合征不会因肿瘤切除而得到解决,因此长期需要通过使用抗生素和静脉注射免疫球蛋白进行强化感染控制来进行仔细管理。对于患有胸腺瘤且疑似易感染的患者,应评估其血清免疫球蛋白水平。

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