Andrews B T, Stricker R B, Kitt D, Galanakis D K, Gerson L B, Hieshima G B
Department of Neurosurgery, California Pacific Medical Center, San Francisco.
Am J Med Sci. 1994 Aug;308(2):106-9. doi: 10.1097/00000441-199408000-00008.
The authors describe a young man with hemophilia complicated by chronic hepatic dysfunction, hypodysfibrinogenemia, and immune thrombocytopenia that resulted in a complex coagulopathy. The patient had a ruptured occipital arteriovenous malformation. The malformation was managed by temporary correction of the coagulopathy using cryoprecipitate, platelet transfusions, and plasmapheresis with fresh frozen plasma replacement. The patient underwent staged preoperative embolization followed by surgical excision of the lesion. Hemostasis was acceptable during the neurointerventional and subsequent surgical management, and no complications of coagulopathy occurred. Plasmapheresis may provide effective preparation for patients with hemophilia and complex coagulation abnormalities who require neurosurgical intervention.
作者描述了一名患有血友病并伴有慢性肝功能不全、低纤维蛋白原血症和免疫性血小板减少症的年轻男性，这些病症导致了复杂的凝血病。该患者患有枕部动静脉畸形破裂。通过使用冷沉淀、血小板输注以及用新鲜冷冻血浆替代进行血浆置换来临时纠正凝血病，从而对该畸形进行处理。患者接受了分期术前栓塞，随后对病变进行手术切除。在神经介入治疗及后续手术治疗过程中止血情况良好，且未发生凝血病并发症。对于需要神经外科干预的血友病和复杂凝血异常患者，血浆置换可能提供有效的术前准备。
