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李××,女,6岁。患儿生后70天开始间断出现皮肤出血点,碰伤后流血不止。有时出现皮下血肿、紫癜,曾有两次咽部大出血,出现失血性休克。4岁后多次出现鼻衄,经输血方可止血。但无深部肌肉及关节出血。家族中无类似出血病史患者。父母非近亲结婚。体检:发育正常,轻度贫血貌。周身皮肤散在淤斑,新旧不一。不高出皮肤,以四肢远端明显。皮肤粘膜无明显毛细血管扩张征象,淋巴结不大,肝脾肋下未触及。
Lee × ×, female, 6 years old. 70 days after birth in children with intermittent skin bleeding point, bleeding after bumps more than. Sometimes there is subcutaneous hematoma, purpura, had two pharyngeal bleeding, hemorrhagic shock. After 4 years of age appear multiple nose epistaxis, bleeding can stop bleeding. But no deep muscle and joint bleeding. There is no history of bleeding in the family. Parents marry non-relatives. Physical examination: normal development, mild anemia appearance. Whole body scattered in the ecchymosis, old and new. Not higher than the skin to distal limbs obvious. Skin and mucosa without obvious signs of telangiectasia, lymph node is not large, liver and spleen ribs did not touch.