title: Splanchnic vein thrombosis in myeloproliferative neoplasms: pathophysiology and molecular mechanisms of disease.
abstract: Myeloproliferative neoplasms (MPNs) are the most common underlying prothrombotic disorder found in patients with splanchnic vein thrombosis (SVT). Clinical risk factors for MPN-associated SVTs include younger age, female sex, concomitant hypercoagulable disorders, and theJAK2V617F mutation. These risk factors are distinct from those associated with arterial or deep venous thrombosis (DVT) in MPN patients, suggesting disparate disease mechanisms. The pathophysiology of SVT is thought to derive from local interactions between activated blood cells and the unique splanchnic endothelial environment. Other mutations commonly found in MPNs, includingCALRandMPL, are rare in MPN-associated SVT. The purpose of this article is to review the clinical and molecular risk factors for MPN-associated SVT, with particular focus on the possible mechanisms of SVT formation in MPN patients.
authors.affiliation: Division of Hematology, Washington University School of Medicine, St Louis, MO, USA.
authors.forename: Joan
authors.initials: J
authors.lastname: How
authors.affiliation: Division of Hematology, Washington University School of Medicine, St Louis, MO, USA.
authors.forename: Amy
authors.initials: A
authors.lastname: Zhou
authors.affiliation: Division of Hematology, Washington University School of Medicine, 660 South Euclid Avenue, Campus Box 8125, St Louis, MO 63110, USA.
authors.forename: Stephen T
authors.initials: ST
authors.lastname: Oh
keywords: Budd-Chiari syndrome; JAK2; essential thrombocythemia; myeloproliferative neoplasm; polycythemia vera; portal vein thrombosis; primary myelofibrosis; splanchnic vein thrombosis