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布加综合征肝移植的血液学方面,特别提及骨髓增殖性疾病

Hematologic aspects of liver transplantation for Budd-Chiari syndrome with special reference to myeloproliferative disorders.

作者信息

Melear Jason M, Goldstein Robert M, Levy Marlon F, Molmenti Ernesto P, Cooper Barry, Netto George J, Klintmalm Goran B, Stone Marvin J

机构信息

Department of Internal Medicine, Baylor University Medical Center, 3500 Gaston Avenue, Dallas, TX 75246, USA.

出版信息

Transplantation. 2002 Oct 27;74(8):1090-5. doi: 10.1097/00007890-200210270-00006.

DOI:10.1097/00007890-200210270-00006
PMID:12438952
Abstract

BACKGROUND

Patients who undergo orthotopic liver transplantation (OLT) for Budd-Chiari syndrome (BCS) traditionally have been anticoagulated with warfarin postoperatively. Because a significant proportion of BCS patients are found to have an underlying myeloproliferative disorder (MPD), antiplatelet therapy may be a more rational treatment strategy for this subgroup.

METHODS

All patients who underwent OLT for the diagnosis of BCS at our institution through March 2000 were included in this analysis. Posttransplant therapy consisted of hydroxyurea and aspirin for those with MPDs. Standard anticoagulation or no antithrombotic treatment was given to BCS patients with other causes. Major posttransplantation complications (thrombosis and bleeding) and mortality were determined.

RESULTS

Seventeen patients underwent OLT for BCS at our institution. The mean follow-up was 68.4 months. Two of seventeen patients died; one patient died of recurrent thrombosis (124 months after OLT) and the other patient died of acute hepatitis B (7 months after OLT). Twelve patients (71%) had evidence of a MPD. Two of the MPD patients were treated with warfarin before the initiation of hydroxyurea and aspirin therapy. The remaining 10 MPD patients were placed on only hydroxyurea and aspirin after OLT. Anagrelide was used in place of hydroxyurea in two patients because of cytopenias caused by the latter agent. The mean follow-up of this group of 10 patients was 59.9 months. Only one patient experienced recurrent thrombosis, which occurred more than 10 years after the original transplant. There were no major bleeding complications and posttransplant liver biopsies were well tolerated.

CONCLUSIONS

Antiplatelet therapy that consists of hydroxyurea and aspirin is a safe and effective alternative to anticoagulation to prevent recurrent thrombosis in MPD patients with BCS after liver transplantation. For patients with a hypercoagulable state corrected by OLT, antithrombotic therapy probably is not required. For those patients with conditions not corrected by OLT or with idiopathic BCS, anticoagulation or other therapy to control the hypercoagulable state should be given.

摘要

背景

传统上,因布加综合征(BCS)接受原位肝移植(OLT)的患者术后一直使用华法林进行抗凝治疗。由于发现相当一部分BCS患者存在潜在的骨髓增殖性疾病(MPD),抗血小板治疗可能是该亚组患者更合理的治疗策略。

方法

纳入在我院截至2000年3月因诊断为BCS而接受OLT的所有患者。对于患有MPD的患者,移植后治疗包括羟基脲和阿司匹林。其他病因的BCS患者给予标准抗凝治疗或不进行抗血栓治疗。确定移植后的主要并发症(血栓形成和出血)及死亡率。

结果

我院有17例患者因BCS接受了OLT。平均随访时间为68.4个月。17例患者中有2例死亡;1例患者死于复发性血栓形成(OLT后124个月),另1例患者死于急性乙型肝炎(OLT后7个月)。12例患者(71%)有MPD证据。2例MPD患者在开始羟基脲和阿司匹林治疗前使用了华法林。其余10例MPD患者在OLT后仅接受羟基脲和阿司匹林治疗。2例患者因羟基脲引起血细胞减少而使用阿那格雷替代羟基脲。这10例患者的平均随访时间为59.9个月。仅1例患者出现复发性血栓形成,发生在初次移植10多年后。未发生严重出血并发症,移植后肝脏活检耐受性良好。

结论

由羟基脲和阿司匹林组成的抗血小板治疗是肝移植后MPD合并BCS患者预防复发性血栓形成的一种安全有效的抗凝替代疗法。对于OLT纠正了高凝状态的患者,可能不需要抗血栓治疗。对于OLT未纠正的疾病患者或特发性BCS患者,应给予抗凝或其他控制高凝状态的治疗。

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