USC Hematology, ASST Papa Giovanni XXIII, Bergamo, Italy.
Institute of Hematology, Catholic University, Roma, Italy.
Blood Cancer J. 2018 Jun 26;8(7):64. doi: 10.1038/s41408-018-0100-9.
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.
骨髓增殖性肿瘤（MPN）是导致脾静脉血栓形成（SVT）的主要原因。SVT 可见于所有 MPN 中，且常影响年轻患者。治疗应针对三个主要目标：预防血栓复发、治疗基础 MPN 以及支持肝功能。长期口服维生素 K 拮抗剂的抗凝治疗是抗血栓治疗的基石。然而，15-20%的患者可能会出现 SVT 或其他血栓复发。直接口服抗凝剂可能是一种替代选择，初步数据鼓励进行比较研究。SVT 患者的生存主要受基础肿瘤的自然史影响，而非 SVT 事件。建议进行积极的治疗管理，并提出了一种基于不同 MPN 亚型的治疗算法。对于真性红细胞增多症和原发性血小板增多症，羟基脲是细胞减灭药物的首选；对于中高危骨髓纤维化和对羟基脲不耐受或耐药的 PV 患者，芦可替尼是首选。MPN 中的 SVT 管理需要多学科方法，可能包括血液科医生、胃肠病学家、介入放射学家和外科医生。尽管进行了药物治疗，但如果患者病情恶化，应考虑对 SVT 患者进行侵袭性操作或肝移植。
