家族型粒细胞减少症伴免疫球蛋白异常是一种罕见的疾病。现报告一例:黄××,男,17个月。住院号:10669。患者于1989年10月16日因发热伴口腔溃疡一周入院。发现粒细胞减少(WBC2.3×10~9/L,N2%,M20%,L74%,E4%)。后因拒绝检查,自动出院。同年12月16日因发热、咳嗽使用青霉素治疗五天无效,因胸片提示肺炎收入院。既往史:自幼经常伤风、咳嗽、发热。个人史:足月顺产,母乳喂养。父母非近亲结婚。其大哥出生后16个月死于肺炎。二哥生 Familial neutropenia with immunoglobulin abnormalities is a rare disease. Now report a case: yellow × ×, male, 17 months. Hospital number: 10669. Patients were admitted to hospital on October 16, 1989 due to fever and oral ulcer. Found neutropenia (WBC 2.3 × 10 ~ 9 / L, N2%, M20%, L74%, E4%). After the refusal to check, discharged automatically. December 16 the same year due to fever, cough penicillin treatment for five days is invalid, because of chest X-ray prompted pneumonia income hospital. Past history: childhood often cold, cough, fever. Personal history: full term spontaneous, breastfeeding. Parents marry non-relatives. His brother died of pneumonia 16 months after birth. Brother students