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[原位肝移植治疗巨大海绵状血管瘤及卡萨巴赫-梅里特综合征]

[Orthotopic liver transplant for giant cavernous hemangioma and Kasabach-Merritt syndrome].

作者信息

Mora A, Cortés C, Roigé J, Noguer M, Camps M A, Margarit C

机构信息

Servicio de Anestesiología y Reanimación, Hospital General Universitario Vall d'Hebron, Barcelona.

出版信息

Rev Esp Anestesiol Reanim. 1995 Feb;42(2):71-4.

PMID:7899656
Abstract

We describe the case of a 42-year-old woman with giant cavernous hemangioma and Kasabach-Merritt syndrome. The patient presented with consumption coagulopathy due to intravascular, intratumoral coagulation as revealed by low platelet levels, fibrinogenopenia and an increase in fibrinolysis with high levels of fibrinogen degradation products. She was scheduled to receive an orthotopic liver transplant because of three factors: respiratory distress caused by compression of the diaphragm by the giant tumor; the risk of bleeding caused by spontaneous rupture or trauma; and the presence of Kasabach-Merritt syndrome due to consumption coagulopathy. Before surgery fibrinogen deficit was corrected with 4 units of cryoprecipitates and low platelet level was treated with 10 units of platelets. Coagulopathy during surgery was corrected with fresh plasma (17 units), cryoprecipitates (6 U), aprotinin (1 x 10(6) U/kg) and antithrombin 3 (2000 U). Blood loss was compensated for with 9 units of packed red blood cells. This report describes the procedures used for anesthesia, for prevention of accidental bleeding during surgery, hemodynamic control and preoperative coagulation testing.

摘要

我们描述了一名患有巨大海绵状血管瘤和卡萨巴赫-梅里特综合征的42岁女性病例。患者因血管内、肿瘤内凝血出现消耗性凝血病,表现为血小板水平降低、纤维蛋白原减少以及纤维蛋白溶解增加,纤维蛋白原降解产物水平升高。由于三个因素,她计划接受原位肝移植:巨大肿瘤压迫膈肌导致呼吸窘迫;自发破裂或外伤引起出血的风险;以及因消耗性凝血病导致的卡萨巴赫-梅里特综合征的存在。手术前,用4单位冷沉淀纠正纤维蛋白原缺乏,用10单位血小板治疗低血小板水平。手术期间的凝血病用新鲜血浆(17单位)、冷沉淀(6单位)、抑肽酶(1×10⁶单位/千克)和抗凝血酶3(2000单位)纠正。用9单位浓缩红细胞补偿失血。本报告描述了麻醉、预防手术期间意外出血、血流动力学控制和术前凝血测试所采用的程序。

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