Good综合征10例临床分析  

Clinical analysis of Good＇s syndrome in 10 cases

作　　者：王艳侠[1] 田新平[1] 张烜[1] 赵岩[1] 董怡[1] 

机构地区：[1]中国医学科学院 北京协和医学院 北京协和医院风湿免疫科,100032

出　　处：《中华医学杂志》2011年第21期1490-1492,共3页National Medical Journal of China

摘　　要：目的探讨Good综合征（GS）的临床病理特点、诊断和治疗方案的选择。方法回顾性分析北京协和医院2000年1月至2011年1月期间收住院治疗的10例GS患者的临床资料，对其临床表现、辅助检查、治疗及转归情况进行总结分析。结果10例患者中男3例，女7例，发病年龄平均（49±11）岁。以肺部感染（6例）、腹泻（4例）、神经系统受累（4例）及多关节痛（3例）为主要临床表现。10例患者均有低免疫球蛋白血症和淋巴细胞亚群异常，影像学检查示所有患者均存在胸腺瘤。胸腺瘤切除术后无明显疗效，静脉输注丙种球蛋白（IVIG）治疗有效。8例患者得到随访，其中4例死亡，肺部感染和中枢神经系统受累为主要死亡原因；4例患者定期接受IVIG治疗，临床症状好转。结论GS好发于中老年人，临床表现缺乏特异性，胸腺瘤合并免疫缺陷为其主要特征。定期行丙种球蛋白替代治疗是目前改善患者临床症状，降低患者死亡率的最有效方法，但由于多数患者未能及时定期接受IVIG治疗，预后较差。Objective To summarize the clinical characteristics of Good＇s syndrome so as to standardize its diagnostic and therapeutic strategy. Methods Ten cases of Good＇s syndrome treated at our hospital from January 2000 to January 2011 were analyzed retrospectively. Their clinical manifestations, accessory examinations, treatment and prognosis were evaluated. Results There were 3 males and 7 females with an average age of （49 _＋ 11 ） years old. Their major symptoms were pulmonary infection （n = 6）, diarrhea （ n = 4）, involvement of central nervous system （CNS） （ n = 4 ） and polyartieular pain （ n = 3 ）. Hypogammaglobulinaemia and abnormal subsets of lymphoeytes were the most common laboratory changes. Besides imaging studies revealed that all of them were diagnosed as thymoma. No significant efficacy was observed after thymoma resection. Clinical improvements were demonstrated after the treatment with regular intravenously administered immunoglobulin （IVIG） in 4 of them. Eight patients were followed up. Four of them died from pulmonary infection and involvement of CNS. Conclusion As a rare disease in adults, Good＇s syndrome is characterized by thymoma associated with immunodeficieney and a lack of specific clinical manifestations. IVIG at regular intervals is the most effective way of improving the clinical symptoms and reducing the patient mortality. However, the prognosis remains poor since most patients fail to receive a regular therapy of IVIG.

关 键 词：免疫系统疾病 治疗 预后 Good综合征 

分 类 号：R593.3[医药卫生—内科学]