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Good 综合征的不良病例。

A Bad Case of Good's Syndrome.

机构信息

Division of Allergy and Clinical Immunology, UCLA School of Medicine, 1301 20th Street, Suite 220, Santa Monica, CA, 90404, USA.

Division of Internal Medicine, Kaiser Permanente, Downey, CA, USA.

出版信息

Infect Dis Ther. 2014 Dec;3(2):333-7. doi: 10.1007/s40121-014-0045-7. Epub 2014 Oct 7.

Abstract

Good's syndrome is a relatively rare immunodeficiency condition that presents in the fourth or fifth decade of life and is defined by hypogammaglobulinemia in the setting of a thymoma. The humoral defect may be severe enough to cause an absence in B cells, with a consequent recurrence of sinopulmonary disease, chronic non-infectious diarrhea and opportunistic infections. The prognosis in patients with Good's syndrome appears to be worse than in those with X-linked agammaglobulinemia (XLA) and common variable immune deficiency (CVID). There have only been three cases of Good's syndrome associated with mycobacterium, and only one case with a cavitary lesion in the lungs. We present here a unique case of Good's syndrome with a non-mycobacterial cavitary lesion.

摘要

Good 综合征是一种相对罕见的免疫缺陷病,发病于第四或第五个十年,其特征是在胸腺瘤的背景下出现低丙种球蛋白血症。体液缺陷可能严重到导致 B 细胞缺失,从而导致鼻-肺疾病、慢性非传染性腹泻和机会性感染的复发。Good 综合征患者的预后似乎比 X 连锁无丙种球蛋白血症(XLA)和普通可变免疫缺陷(CVID)患者更差。与分枝杆菌相关的 Good 综合征仅有三例,肺部有空腔病变的仅有一例。我们在此介绍一例独特的 Good 综合征伴非分枝杆菌性空洞病变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/60c5/4269629/8b38b7595c20/40121_2014_45_Fig1_HTML.jpg

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