Budd–Chiari Syndrome and Myeloproliferative Neoplasms

  • Valerio De StefanoEmail author
  • Elena Rossi


Non-cirrhotic and non-malignant splanchnic vein thrombosis (SVT) recognizes Philadelphia-negative myeloproliferative neoplasms (MPN) as the most frequent systemic cause. An overt MPN is diagnosed in 40% of the patients with Budd–Chiari syndrome (BCS). In BCS patients, the MPN molecular hallmark JAK2 V617F is present in up to 80% of those with overt MPN and up to 43% of those without an overt diagnosis according to the WHO criteria. In those latter, the other MPN driver mutations in the JAK2 exon 12, CALR, and MPL genes are infrequent.

Treatment of the acute phase of BCS does not differ from that employed in non-MPN patients and is based on immediate anticoagulation with heparin, together with endovascular treatment with a transjugular intrahepatic portosystemic shunt and/or angioplasty/stenting. In the case of no response to such treatments, liver transplantation is the only reliable option for treatment of BCS, and the presence of MPN does not influence the survival outcome. Indefinite treatment with oral anticoagulation based on vitamin K-antagonists is recommended in all BCS patients. Cytoreduction is warranted in all MPN patients with thrombosis, but its efficacy in preventing recurrent thromboses is doubtful in the patients with SVT.


Budd–Chiari syndrome Myeloproliferative neoplasms JAK2 V617F mutation CALR mutations MPL mutations Recurrent venous thrombosis Antithrombotic treatment Vitamin-k antagonists Hydroxyurea 


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Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Istituto di Ematologia, Università CattolicaRomaItaly
  2. 2.Fondazione Policlinico A. Gemelli IRCCSRomaItaly

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