Cruz Elizabeth, Ascher Nancy L, Roberts John P, Bass Nathan M, Yao Francis Y
Division of Gastroenterology, Department of Medicine, University of California, San Francisco, CA 94143-0538, USA.
Clin Transplant. 2005 Aug;19(4):501-6. doi: 10.1111/j.1399-0012.2005.00374.x.
Most cases of Budd-Chiari syndrome (BCS) in Western countries are related to underlying hematologic diseases with inherent thrombogenic propensity. We evaluated the long-term outcome, risks for recurrent disease, and other hematologic complications following orthotopic liver transplantation (OLT) for BCS.
Clinical data from 11 consecutive patients with BCS who underwent OLT were retrospectively reviewed. Four patients had a prior transjugular intrahepatic portosystemic shunt and one had a surgical shunt procedure. All patients were started on intravenous heparin within the first 24 h following OLT. All except one patient who had protein C deficiency were maintained on long-term oral anticoagulation.
The Kaplan-Meier survival rates at 1, 5 and 10 yr were 81, 65 and 65%, respectively. Three patients developed BCS recurrence, including two who died as a consequence of rapid graft failure within days after OLT. Three patients developed other thrombotic events, including splenic vein thrombosis associated with gastric variceal hemorrhage requiring splenectomy, portal vein thrombosis and pulmonary embolism. Four patients experienced severe bleeding complications within 7 d after OLT requiring exploratory laparotomy. One patient died after transformation of polycythemia vera to acute myelogenous leukemia at 2.1 yr after OLT.
We observed a high incidence of recurrent BCS and complications related to the underlying hematologic disorder or anticoagulation after OLT for BCS. The present series also included the first two cases of rapid recurrence of BCS and graft failure within days after OLT.
西方国家大多数布加综合征（BCS）病例与具有内在血栓形成倾向的潜在血液系统疾病有关。我们评估了原位肝移植（OLT）治疗BCS后的长期预后、疾病复发风险及其他血液系统并发症。
回顾性分析11例连续接受OLT治疗的BCS患者的临床资料。4例患者曾接受经颈静脉肝内门体分流术，1例曾接受外科分流手术。所有患者在OLT术后24小时内开始静脉注射肝素。除1例蛋白C缺乏患者外，所有患者均长期口服抗凝药。
1年、5年和10年的Kaplan-Meier生存率分别为81%、65%和65%。3例患者发生BCS复发，其中2例在OLT术后数天内因移植肝快速衰竭死亡。3例患者发生其他血栓事件，包括与胃静脉曲张出血相关的脾静脉血栓形成（需行脾切除术）、门静脉血栓形成和肺栓塞。4例患者在OLT术后7天内发生严重出血并发症，需行剖腹探查术。1例患者在OLT术后2.1年真性红细胞增多症转化为急性髓系白血病后死亡。
我们观察到OLT治疗BCS后BCS复发以及与潜在血液系统疾病或抗凝相关并发症的发生率较高。本系列还包括OLT术后数天内BCS快速复发和移植肝衰竭的首例2例病例。
