普通变异型免疫缺陷病并发非肝硬化性门脉高压症6例临床资料分析  

Clinical features of non-cirrhotic portal hypertension in patients with common variable immunodeficiency

作　　者：武娟 韩欣欣 狄虹 尹月 韩迎东 王玉 张昀 曾学军 Wu Juan;Han Xinxin;Di Hong;Yin Yue;Han Yingdong;Wang Yu;Zhang Yun;Zeng Xuejun(Department of Family Medicine&Division of General Internal Medicine,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,State Key Laboratory of Complex Severe and Rare Diseases,Beijing 100730,China)

机构地区：[1]中国医学科学院、北京协和医学院、北京协和医院全科医学科(普通内科)疑难重症及罕见病国家重点实验室,北京100730

出　　处：《中华内科杂志》2023年第8期1007-1011,共5页Chinese Journal of Internal Medicine

基　　金：国家自然科学基金(81901667);北京协和医学院2019年学科建设项目(201920200106)。

摘　　要：分析总结普通变异型免疫缺陷病(CVID)并发非肝硬化性门脉高压症(NCPH)的临床特征,提高对其的认识水平。回顾分析北京协和医院1983年1月至2021年5月收治的6例CVID合并NCPH的病例资料,总结其临床特点。结果显示,6例CVID合并NCPH患者,男女各3例,起病年龄16~45岁,4例以门脉高压表现起病,表现为贫血、脾大,2例以反复感染起病。4例患者合并自身免疫病。2例患者完善肝活检,均诊断为肝结节性再生性增生(NRH)。6例患者均予静脉注射免疫球蛋白G 0.4~0.6 g/kg、每3~4周1次作为基础治疗。2例患者行脾切除术,部分改善血液系统异常。CVID并发NCPH常以门脉高压的表现为首发症状,常有自身免疫相关表现。影像学筛查可提供重要诊断线索。究其病因,可能与肝结节性再生性增生、反复感染所致的脾大相关。We wished to summarize the clinical features of common variable immunodeficiency(CVID)complicated by non-cirrhotic portal hypertension(NCPH)and to deepen our understanding of it.The case data of CVID complicated with NCPH admitted to Peking Union Medical College Hospital from January 1983 to May 2021 were analyzed retrospectively to summarize their clinical characteristics.Six patients with CVID combined with NCPH(three of each sex;16-45 years)were assessed.Four patients had portal hypertension.All patients had anemia,splenomegaly,a normal serum level of albumin and transaminases,and possibly increased levels of alkaline phosphatase and gamma-glutamyl transpeptidase.Two patients were diagnosed with esophagogastric fundic varices by gastroscopy.Two patients underwent splenectomy(which improved hematologic abnormalities partially).Four patients had autoimmune disease.Two cases were diagnosed with nodular regenerative hyperplasia(NRH)upon liver biopsy.Six patients were administered intravenous immunoglobulin-G(0.4-0.6 g/kg bodyweight)once every 3-4 weeks as basic therapy.Often,CVID complicated with NCPH has:(1)The manifestations of portal hypertension as the primary symptom.(2)Autoimmune-related manifestations.Imaging can provide important diagnostic clues.The etiology may be related to hepatic NRH and splenomegaly due to recurrent infections.

关 键 词：普通变异型免疫缺陷病 高血压 门静脉 非肝硬化 

分 类 号：R575.2[医药卫生—消化系统] R593.3[医药卫生—内科学]