Service de rhumatologie, centre hospitalier René-Dubos, 6, avenue de l'Île-de-France, BP 79, 95303 Cergy-Pontoise cedex, France.
Joint Bone Spine. 2012 Jul;79(4):412-4. doi: 10.1016/j.jbspin.2012.03.009. Epub 2012 May 9.
We report on a 46-year-old woman, who developed streptococcus B septic polyarthritis in the context of septicemia of urinary origin. This case revealed a Good's syndrome whose diagnosis was made on the basis of a profound hypogammaglobulinaemia, a large decrease of peripheral B cells and a thymoma disclosed on chest computed tomography (CT) and confirmed by surgical removal (AB type). There was also an inversion of the peripheral CD4+/CD8+ T cell ratio and an increase of CD8+ T cells. The course of infection was favorable under treatment with antibiotics and intravenous immunoglobulin. Good's syndrome is a rare entity, which belongs to primary immunodeficiency syndromes. Its first manifestations appear late in life, usually after the age of 40. In front of hypogammaglobulinemia, it is necessary to search for Good's syndrome by practicing chest CT scan, looking for thymoma, which is constant, and peripheral lymphocytes phenotyping looking for B cell lymphopenia and the frequent T cell associated abnormalities.
我们报告了一例 46 岁女性,在尿路感染引起的败血症背景下发生乙型链球菌脓毒性多关节炎。该病例揭示了一种 Good 综合征,其诊断基于严重的低丙种球蛋白血症、外周 B 细胞大量减少以及胸部计算机断层扫描 (CT) 显示的胸腺瘤,并通过手术切除 (AB 型) 得到证实。外周 CD4+/CD8+ T 细胞比值也发生倒置,CD8+ T 细胞增加。在抗生素和静脉注射免疫球蛋白治疗下,感染过程良好。Good 综合征是一种罕见的实体,属于原发性免疫缺陷综合征。其首次表现出现在生命后期,通常在 40 岁以后。在低丙种球蛋白血症的情况下,有必要通过进行胸部 CT 扫描来寻找 Good 综合征,寻找恒定的胸腺瘤,以及外周淋巴细胞表型寻找 B 细胞淋巴细胞减少和常见的 T 细胞相关异常。