患儿男 ,5 5岁。因反复皮肤瘀斑、鼻衄 5年余 ,加重 2天入院。生后 5个月患儿不明原因出现皮肤瘀斑、皮下血肿及鼻衄 ,不伴呕血、便血及血尿 ,病情反复。入院 2天前患儿剧烈活动后再次发病 ,并呈进行性贫血 ,烦躁 ,嗜睡 ,尿少 ,急诊入院。父母体健 ,非近亲结婚 ,家族中无出血 Children male, 5 5 years old. Due to repeated skin ecchymosis, epistaxis more than 5 years, increased 2 days admission. 5 months after birth, children with unexplained skin ecchymosis, subcutaneous hematoma and epistaxis, without hematemesis, hematochezia and hematuria, repeated illness. Admission 2 days before the onset of severe activity in children again, and was progressive anemia, irritability, drowsiness, oliguria, emergency admission. Healthy parents, non-relatives married, no bleeding in the family