Myeloproliferative
neoplasms (MPNs) are a leading cause of splanchnic vein
thrombosis (SVT). SVT is observed in all MPNs and frequently affects young patients.
Therapy should be addressed to three main goals: preventing
thrombosis recurrence, managing the underlying MPN, and supporting
liver dysfunction. Life-long oral anticoagulation with
vitamin K antagonists is the cornerstone of the antithrombotic treatment. However, recurrences of SVT or other
thrombosis may occur in 15-20% of patients. Direct oral
anticoagulants can represent an alternative and preliminary data encourage comparative studies. Survival of patients with SVT in MPN is primarily influenced by the natural history of the underlying
neoplasms, rather than the SVT event. An aggressive management is recommended and a treatment algorithm based on the different MPN subtypes is proposed.
Hydroxyurea is the cytoreductive drug of choice in
polycythemia vera and
essential thrombocythemia, whereas
ruxolitinib is indicated in intermediate and high-risk patients with
myelofibrosis and in PV patients resistant or intolerant to
hydroxyurea. The management of SVT in MPNs requires a multidisciplinary approach that may include a hematologist, a gastroenterologist, an interventional radiologist, and a surgeon. In the case of
clinical deterioration despite pharmacological
therapy, patients with SVT should be considered for invasive procedures or
liver transplantation.