Splanchnic vein thrombosis associated with myeloproliferative neoplasms

Abstract

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.