D. Bezinover, S. Sugino, Yuka Imamura-Kawasawa, M. S. Bell, Z. Kadry, P. Janicki
{"title":"肝移植过程中大量暴发性血栓形成患者先前未知的抗凝血酶途径突变。","authors":"D. Bezinover, S. Sugino, Yuka Imamura-Kawasawa, M. S. Bell, Z. Kadry, P. Janicki","doi":"10.1213/XAA.0000000000000396","DOIUrl":null,"url":null,"abstract":"We describe a case of fulminant intraoperative thrombosis during deceased donor liver transplantation. Despite significant medical bleeding, the patient suddenly developed diffuse thrombosis in all chambers of the heart and pulmonary vasculature resulting in intraoperative death. The patient's postmortem genetic analysis demonstrated a deleterious missense mutation in a coagulation pathway gene, SERPINC1, which codes for antithrombin III. The level of antithrombin III was not available to directly prove the causality of thrombosis, but our findings suggest that this mutation, in combination with antifibrinolytic administration in a hypercoagulable cirrhotic patient, might have contributed to the development of this catastrophic thrombotic event.","PeriodicalId":6824,"journal":{"name":"A&A Case Reports ","volume":"36 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2016-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Massive Fulminant Thrombosis During Liver Transplantation in a Patient With a Previously Unknown Antithrombin Pathway Mutation.\",\"authors\":\"D. Bezinover, S. Sugino, Yuka Imamura-Kawasawa, M. S. Bell, Z. Kadry, P. Janicki\",\"doi\":\"10.1213/XAA.0000000000000396\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"We describe a case of fulminant intraoperative thrombosis during deceased donor liver transplantation. Despite significant medical bleeding, the patient suddenly developed diffuse thrombosis in all chambers of the heart and pulmonary vasculature resulting in intraoperative death. The patient's postmortem genetic analysis demonstrated a deleterious missense mutation in a coagulation pathway gene, SERPINC1, which codes for antithrombin III. The level of antithrombin III was not available to directly prove the causality of thrombosis, but our findings suggest that this mutation, in combination with antifibrinolytic administration in a hypercoagulable cirrhotic patient, might have contributed to the development of this catastrophic thrombotic event.\",\"PeriodicalId\":6824,\"journal\":{\"name\":\"A&A Case Reports \",\"volume\":\"36 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2016-12-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"A&A Case Reports \",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1213/XAA.0000000000000396\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"A&A Case Reports ","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1213/XAA.0000000000000396","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Massive Fulminant Thrombosis During Liver Transplantation in a Patient With a Previously Unknown Antithrombin Pathway Mutation.
We describe a case of fulminant intraoperative thrombosis during deceased donor liver transplantation. Despite significant medical bleeding, the patient suddenly developed diffuse thrombosis in all chambers of the heart and pulmonary vasculature resulting in intraoperative death. The patient's postmortem genetic analysis demonstrated a deleterious missense mutation in a coagulation pathway gene, SERPINC1, which codes for antithrombin III. The level of antithrombin III was not available to directly prove the causality of thrombosis, but our findings suggest that this mutation, in combination with antifibrinolytic administration in a hypercoagulable cirrhotic patient, might have contributed to the development of this catastrophic thrombotic event.