Pediatrics, University of Kentucky, Lexington, Kentucky, USA
Pediatrics, University of Kentucky, Lexington, Kentucky, USA.
BMJ Case Rep. 2021 Aug 17;14(8):e241613. doi: 10.1136/bcr-2021-241613.
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.
一名 18 岁男性，患有 3 型血管性血友病（VWD），出现自发性血胸。3 型 VWD 可能表现为黏膜皮肤和深部出血，如内脏出血、颅内出血和关节积血。在儿童中，自发性血胸的病例非常少见。由于手术引流后出血的风险，该患者的治疗极具挑战性，需要持续输注血管性血友病因子浓缩物进行替代治疗，同时监测 VIII 因子水平以平衡血栓形成的风险。
