文章：

骨髓增殖性肿瘤中的内脏静脉血栓形成：2018年治疗流程

Splanchnic vein thrombosis in myeloproliferative neoplasms: treatment algorithm 2018

原文发布日期：2018-06-26

DOI: 10.1038/s41408-018-0100-9

类型: Current Treatment Algorithm

开放获取: 是

英文摘要：

Myeloproliferative neoplasms (MPNs) are a leading cause of splanchnic vein thrombosis (SVT). SVT is observed in all MPNs and frequently affects young patients. Therapy should be addressed to three main goals: preventing thrombosis recurrence, managing the underlying MPN, and supporting liver dysfunction. Life-long oral anticoagulation with vitamin K antagonists is the cornerstone of the antithrombotic treatment. However, recurrences of SVT or other thrombosis may occur in 15–20% of patients. Direct oral anticoagulants can represent an alternative and preliminary data encourage comparative studies. Survival of patients with SVT in MPN is primarily influenced by the natural history of the underlying neoplasms, rather than the SVT event. An aggressive management is recommended and a treatment algorithm based on the different MPN subtypes is proposed. Hydroxyurea is the cytoreductive drug of choice in polycythemia vera and essential thrombocythemia, whereas ruxolitinib is indicated in intermediate and high-risk patients with myelofibrosis and in PV patients resistant or intolerant to hydroxyurea. The management of SVT in MPNs requires a multidisciplinary approach that may include a hematologist, a gastroenterologist, an interventional radiologist, and a surgeon. In the case of clinical deterioration despite pharmacological therapy, patients with SVT should be considered for invasive procedures or liver transplantation.

摘要翻译： 

骨髓增殖性肿瘤（MPNs）是内脏静脉血栓（SVT）的主要病因。所有类型的MPNs均可能并发SVT，且常见于年轻患者。治疗应着眼于三个主要目标：预防血栓复发、控制潜在MPN及支持肝功能治疗。终身口服维生素K拮抗剂抗凝是抗血栓治疗的基石。但仍有15%-20%的患者可能出现SVT复发或其他血栓事件。直接口服抗凝药可作为替代选择，初步数据支持开展对比研究。MPN合并SVT患者的生存率主要受潜在肿瘤自然病程影响，而非SVT事件本身。建议采取积极管理策略，并根据不同MPN亚型提出治疗流程。羟基脲是真性红细胞增多症和原发性血小板增多症的首选细胞减灭药物，而鲁索替尼适用于中高危骨髓纤维化患者以及对羟基脲耐药或不耐受的真红患者。MPNs合并SVT的管理需要多学科协作，团队可包括血液科医师、消化科医师、介入放射科医师和外科医师。若药物治疗后出现临床恶化，应考虑对SVT患者实施介入治疗或肝移植。

原文链接：

Splanchnic vein thrombosis in myeloproliferative neoplasms: treatment algorithm 2018