Splanchnic vein thrombosis associated with myeloproliferative neoplasms. Issue 218 (October 2022)

• Record Type: Journal Article
• Authors: Tremblay, Douglas; Winters, Adam; Beckman, Joan D.; Naymagon, Leonard; Patel, Rahul; Mascarenhas, John; Schiano, Thomas D.
• Is Part Of: Thrombosis research. Issue 218(2022)
• Page Start: 8
• Page End: 16
• Publication Date: 2022-10
• Subjects: Splanchnic vein -- Thrombosis -- Myeloproliferative -- Polycythemia vera -- Essential thrombocythemia -- Myelofibrosis; Thrombosis -- Periodicals; 616.135
• DOI: 10.1016/j.thromres.2022.08.003
• Languages: English
• ISSNs: 0049-3848

Abstract

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. Highlights: Splanchnic vein thrombosis can be a manifestation of a myeloproliferative neoplasm. Endothelial cells play a major role in the pathogenesis of MPN-related SVT. Anticoagulation is the mainstay of therapy and direct oral anticoagulants are safe. Endovascular procedures, such as transjugular intrahepatic portosystemic shunt, should be considered in select patients. Although recommended, the role of cytoreductive therapy is not well established.