原发性免疫缺陷病(primary immunodeficiency diseases,PID)是一组由遗传学因素引起的免疫功能缺失或减低的临床综合征,以易患感染、肿瘤、自身免疫病为特点。我院近期诊治了1例PID继发噬血细胞综合征的患者,现将该患者的临床资料报告如下。1病例资料患者,男,15岁,2014年4月24日因“间断发热、乏力4个月”入院。患者于入院前4个月无明显诱因出现发热,体温最高达40℃,伴头痛、流清 Primary immunodeficiency diseases (PIDs) are a group of clinical syndromes characterized by loss of or loss of immune function due to genetic factors and are characterized by predisposition to infection, oncology and autoimmune diseases. Our hospital recently diagnosed a case of PID secondary hemophagocytic syndrome patients, now the clinical data of the patient reported as follows. 1 case data patient, male, 15 years old, April 24, 2014 due to “intermittent fever, fatigue for 4 months ” admission. Patients in the 4 months before admission no obvious incentive to fever, body temperature up to 40 ℃, with headache, fluid