23例原发性免疫缺陷病接种卡介苗后发生播散性卡介菌病的临床特征及疗效分析

Clinical characteristics and therapeutic effect analysis of 23 cases of disseminated BCG disease after BCG vaccination caused by primary immunodeficiency disease

目的探讨原发性免疫缺陷病接种卡介苗后发生播散性卡介菌病的临床特点、免疫学特点及治疗效果。方法收集2015年1月至2019年12月重庆医科大学附属儿童医院收治的23例原发性免疫缺陷病接种卡介苗后发生播散性卡介菌病的患儿。其中,男18例,女5例,均在接种后6个月内出现播散性卡介菌病。对其临床资料及免疫学特征进行分析,并随访患儿的治疗效果。结果23例患儿中,慢性肉芽肿病16例,原发性免疫缺陷病3例,重症联合免疫缺陷病2例,X连锁重症联合免疫缺陷病1例,X连锁高IgM综合征1例;23例均发生肺部播散,3例发生肝脏播散,1例发生腹腔播散,1例发生左侧股骨播散,1例发生颅内播散。16例慢性肉芽肿病患儿的白细胞吞噬功能试验均异常;1例X连锁高IgM综合征患儿免疫球蛋白水平测定IgM水平明显升高,伴补体C3水平下降;2例重症联合免疫缺陷病患儿淋巴细胞分类检测提示CD3^+CD4^+T细胞和CD3^+CD8^+T细胞明显降低,伴免疫球蛋白水平下降。23例患儿中,死亡6例,病死率为26.1%;17例存活患儿随访1.0~2.8年,处于间断住院治疗中。结论原发性免疫缺陷病接种卡介苗后发生播散性卡介菌病起病较早,慢性肉芽肿病在此类患儿中较为常见;此病病死率高,预后不良,需明确免疫缺陷病的类型并尽早行针对性治疗。

Objective To investigate the clinical characteristics,immunological characteristics and therapeutic effects of disseminated BCG complicated with primary immunodeficiency disease.Methods Twentythree patients with disseminated BCG complicated with primary immunodeficiency disease were retrospectively collected from January 2015 to December 2019 in Children’s Hospital Affiliated to Chongqing Medical University.Among them,there were 18 males and 5 females with disseminated BCG disease within 6 months after inoculation.The clinical data and immunological characteristics were analyzed,and the therapeutic effect was followed up.Results Among the 23 children with disseminated BCG disease after BCG vaccination,there were 16 cases of chronic granulomatosis,3 cases of primary immunodeficiency disease,2 cases of severe combined immunodeficiency disease,1 case of X-linked severe combined immunodeficiency disease and 1 case of X-linked high IgM syndrome.Among the 23 cases,23 cases had pulmonary dissemination,3 cases had liver spread,1 case had abdominal dissemination,1 case had left femur spread and 1 case had intracranial spread.Sixteen cases of children with chronic granulomatosis were abnormal in leukocyte phagocytosis test.One case of X-linked high IgM syndrome had significantly increased IgM level and decreased complement C3 level;two cases of severe combined immunodeficiency disease showed that CD3^+CD4^+T cells and CD3^+CD8^+T cells were significantly decreased,with the decrease of immunoglobulin level.Among the 23 cases,6 cases died,the mortality rate was 26.1%;seventeen cases of survival children were followed up for 1.0-2.8 years and were in intermittent hospitalization.Conclusion Disseminated BCG complicated with primary immunodeficiency has an early onset,and chronic granulomatous disease is more common in these children;this disease has a high mortality rate and poor prognosis,so it is necessary to clarify the type of immunodeficiency and carry out targeted treatment as early as possible.