患儿男性,第一胎,足月顺产。出生后1周因颈部皱褶处皮肤潮红、脐及脐周皮肤红肿首次入院。体检:营养佳,发育正常,五官端正,颈部及脐部患处均未见分泌物及脓肿形成,心肺肝脾均无异常发现。血象(多次检查):白细胞总数7,400～19,800,中性粒细胞明显减少,在0～6%之间,淋巴细胞41～72%,单核细胞7～53%,嗜酸性细胞1～28%,各种白细胞形态无特殊,未见幼稚细胞。血红蛋白15～13克,红细胞440～520万,网织红细胞0.6～1.1%,血小板27～30万。血清免疫球蛋白定量正常,E玫瑰花结试验31%。外周血染色体核型正常。骨髓象:增生活跃,粒:红=0.87:1。早幼粒占11.75%,嗜中性中幼粒、晚幼粒及杆状核细胞分别占0.25%、0.25%和2%,未见中性分叶核细胞;红细胞增生活跃;单核细胞、嗜酸性细胞和巨核细胞比例基本正常;淋巴细胞比例增高。各系细胞形态正常。入院后应用抗生素治疗期间又出现肛周炎合并肛痿,治疗2个月才基本控制感染而出院。1个月后又因患支气管肺炎再次入 Children with children, the first child, full-term delivery. One week after birth, the skin of the folds of the neck is flushed and the umbilical and umbilical skin are inflamed for the first time. Physical examination: good nutrition, normal development, facial features, neck and umbilical area at no signs of secretions and abscesses, heart and lung no abnormal liver and spleen found. Blood tests (multiple examinations): The total number of leukocytes is 7,400 ~ 19,800, the number of neutrophils is obviously decreased, ranging from 0 to 6%, lymphocytes 41 to 72%, monocytes 7 to 53%, eosinophils 1 to 28% , All kinds of white blood cells no special shape, no naive cells. 15 to 13 grams of hemoglobin, 4.4 to 5.2 million red blood cells, reticulocytes 0.6 to 1.1%, and platelets 27 to 300,000. Serum immunoglobulin normal, E rosette test 31%. The karyotype of peripheral blood is normal. Bone marrow: hyperplasia active, grain: red = 0.87: 1. Promyelocytic accounted for 11.75%, neutrophilic juvenile, late promyelocytic and mononuclear cells accounted for 0.25%, 0.25% and 2%, no neutral leaf cells; erythrocyte hyperplasia; monocytes, The proportion of eosinophils and megakaryocytes was basically normal; the proportion of lymphocytes increased. The Department of normal cell morphology. Application of antibiotics during admission appeared perianitis with anal impotence, the treatment of 2 months before the basic control of infection and discharged. 1 month later due to bronchial pneumonia again into