Splanchnic Vein Thrombosis in Myeloproliferative Neoplasms: Treatment Considerations and Unmet Needs

SIMPLE SUMMARY: Splanchnic vein thromboses (SVTs) are atypical clots associated with myeloproliferative neoplasms (MPNs). However, there are no well-established guidelines on how to treat them. The aim of this review is to explore treatment considerations of SVT in the setting of MPN (MPN-SVT). Anticoagulation is the cornerstone of therapy and cytoreductive therapy is recommended per MPN treatment guidelines. Endovascular intervention may also be considered in patients with occlusive or extensive clot burden in the acute setting to prevent or mitigate potential portal hypertensive complications. Beyond these general approaches, there are still gaps in our knowledge of how to treat MPN-SVT, including the optimal dose and timing of anticoagulation, role of endovascular interventions and novel agents, and management of patients with MPN-SVT without elevated counts. Future studies will be needed to bridge the gap of these unmet needs. ABSTRACT: Patients who develop splanchnic vein thrombosis (SVT) in the setting of a myeloproliferative neoplasm (MPN) are at risk for complications including portal hypertension, bleeding, thrombosis, and death. Prompt multidisciplinary treatment is thus necessary to prevent long-term sequelae. However, optimal management strategies are not well established due to a paucity of data. In this review, we very briefly discuss the epidemiology, pathophysiology, and prognosis of MPN-SVT and then more comprehensively explore treatment considerations of MPN-SVT, including anticoagulation, endovascular/surgical intervention, and cytoreductive therapy. We will also highlight current gaps in our knowledge of MPN-SVT and conclude by suggesting future directions to optimize the treatment of MPN-SVT and improve outcomes.