Mo Allison, Testro Adam, French Janine, Robertson Marcus, Angus Peter, Grigg Andrew
Department of Clinical Haematology, Austin Hospital, Melbourne, Victoria, Australia.
Liver Transplant Unit, Austin Hospital, Melbourne, Victoria, Australia.
Intern Med J. 2017 Dec;47(12):1361-1367. doi: 10.1111/imj.13544.
Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments, including pharmacological, radiological and surgical interventions, have been used.
To describe retrospectively our institution's experience with management of patients with BCS.
A retrospective study of all cases of primary Budd-Chiari syndrome presenting to our institution between January 2000 and August 2012 was performed. Patients with secondary Budd-Chiari syndrome due to malignancy or local mass compression were excluded.
Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1.16 (range: 0.07-2.11). A total of 24 patients (89%) had at least one risk factor, with the commonest being myeloproliferative neoplasm (MPN), detected in 17 of 24 (71%) of the tested patients, including four patients with normal blood counts at diagnosis. All patients were anticoagulated with warfarin or low-molecular-weight heparin (LMWH). A total of 25 (92.6%) patients also had primary radiological interventions, consisting of transjugular intrahepatic portosystemic shunt (TIPS) in 18 (67%) patients and/or angioplasty/stenting in 11 (40%). A total of 14 patients developed TIPS stenoses, requiring a median of 1.5 (range: 1-14) revisions. No patient developed TIPS failure requiring alternative therapy. Two patients were lost to follow-up. At a median follow up of 59 months (range: 2-248 months), the overall survival was 96% at 1 year and 81% at 5 years, much greater than predicted by the Rotterdam score. No patients required liver transplantation.
There is a high incidence of MPN in patients with primary BCS, including patients with normal peripheral blood counts at the time of diagnosis. Our approach of anticoagulation, aggressive and early radiological intervention aimed at rapid decompression of the congested liver resulted in excellent medium-term outcomes.
布加综合征（BCS）是一种罕见且危及生命的疾病，由肝静脉血栓形成所致。已采用了包括药物、放射和外科干预在内的各种治疗方法。
回顾性描述我们机构对布加综合征患者的管理经验。
对2000年1月至2012年8月期间在我们机构就诊的所有原发性布加综合征病例进行回顾性研究。排除因恶性肿瘤或局部肿块压迫导致的继发性布加综合征患者。
2000年至2012年期间，27例原发性布加综合征患者的鹿特丹评分中位数为1.16（范围：0.07 - 2.11）。共有24例患者（89%）至少有一个危险因素，最常见的是骨髓增殖性肿瘤（MPN），在24例检测患者中的17例（71%）中检测到，其中包括4例诊断时血常规正常的患者。所有患者均接受华法林或低分子肝素（LMWH）抗凝治疗。共有25例（92.6%）患者还接受了主要的放射学干预，其中18例（67%）患者接受了经颈静脉肝内门体分流术（TIPS）和/或11例（40%）患者接受了血管成形术/支架置入术。共有14例患者出现TIPS狭窄，需要进行中位数为1.5次（范围：1 - 14次）的修复。没有患者出现需要替代治疗的TIPS功能障碍。2例患者失访。在中位随访59个月（范围：2 - 248个月）时，1年总生存率为96%，5年为81%，远高于鹿特丹评分预测的生存率。没有患者需要肝移植。
原发性布加综合征患者中MPN的发生率较高，包括诊断时外周血常规正常的患者。我们采用的抗凝、积极早期放射学干预以迅速减轻肝脏充血的方法取得了良好的中期效果。
