Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, United States of America.
Division of Liver Diseases, Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY, United States of America.
Thromb Res. 2022 Oct;218:8-16. doi: 10.1016/j.thromres.2022.08.003. Epub 2022 Aug 6.
Splanchnic vein thrombosis (SVT) in the setting of myeloproliferative neoplasm (MPN) is a unique clinical entity that requires close interdisciplinary coordination for proper diagnosis and management. The pathobiology of MPN-SVT is not fully understood, but recent developments have revealed the central role of endothelial cells. In this multidisciplinary review, we summarize the epidemiology of MPN-SVT and then critically evaluate the pathogenic features of this complication, with a focus on endothelial cell biology. We then discuss diagnostic considerations, including imaging modalities and MPN-specific investigations. Finally, we critically review the evidence supporting clinical management of MPN-SVT, including anticoagulation, interventional radiology procedures, MPN-related therapies, and liver transplantation. We conclude that further studies are needed to improve our understanding of MPN-SVT and the outcomes of patients with this debilitating complication.
内脏静脉血栓形成（SVT）在骨髓增殖性肿瘤（MPN）的背景下是一种独特的临床实体，需要密切的跨学科协调以进行正确的诊断和管理。MPN-SVT 的病理生物学尚未完全理解，但最近的研究揭示了内皮细胞的核心作用。在这个多学科综述中，我们总结了 MPN-SVT 的流行病学，然后批判性地评估了这种并发症的发病特征，重点关注内皮细胞生物学。然后，我们讨论了诊断注意事项，包括成像方式和 MPN 特异性研究。最后，我们批判性地回顾了支持 MPN-SVT 临床管理的证据，包括抗凝、介入放射学程序、MPN 相关治疗和肝移植。我们得出结论，需要进一步的研究来提高我们对 MPN-SVT 的理解以及患有这种使人衰弱的并发症的患者的结局。
