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.
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.
Keywords
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Article info
Publication history
Published online: August 05, 2022
Accepted:
August 3,
2022
Received in revised form:
June 30,
2022
Received:
May 11,
2022
Identification
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