PDF(Pubmed)
Abstract：
An 18-year-old man with a history of type 3 von Willebrand disease (VWD) presented with a spontaneous pyohaemothorax. Type 3 VWD may present with both mucocutaneous and deep-seated bleeds, such as visceral haemorrhages, intracranial bleeds and haemarthrosis. There have been very few cases described in children of spontaneous pyohaemothorax. Management of this patient was challenging due to risks of bleeding following surgical drainage, requiring constant replacement with von Willebrand factor concentrate, while monitoring factor VIII levels to balance the risks of thrombosis.
摘要：
一名有3型血管性血友病（VWD）病史的18岁男子表现为自发性脓性血胸。3型VWD可能同时伴有皮肤粘膜出血和深层出血，例如内脏出血，颅内出血和关节积血。在自发性脓性血胸儿童中描述的病例很少。由于手术引流后出血的风险，该患者的管理具有挑战性。需要不断更换vonWillebrand因子浓缩物，同时监测因子VIII水平以平衡血栓形成的风险。
