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Budd-Chiari Syndrome and Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome
  1. Dominique-Charles Valla (dominique.valla{at}bjn.ap-hop-paris.fr)
  1. Hopital Beaujon, France

    Abstract

    Budd-Chiari syndrome (BCS) · Many patients with BCS harbour several prothrombotic disorders. · Liver disease blurs the diagnostic features of most prothrombotic disorders. · Myeloproliferative disease account for 50% of BCS patients. Their recognition largely relies on the assessment of V617F JAK2 mutation in peripheral blood. · Treatment for BCS consists of the successive implementation of anticoagulation, percutaneous angioplasty, transjugular intrahepatic shunt and last, liver transplantation. · Using this progressive treatment strategy, overall 5-year survival rates approaching 90% are currently achieved. Veno-occlusive disease/Sinusoidal obstruction syndrome (VOD/SOS) · VOD/SOS is produced by a toxic injury to sinusoidal endothelial cells. · Many chemotherapeutic agents can induce VOD/SOS. · Knowledge in the metabolic determinants of drug toxicity to sinusoidal endothelium has deepened. The translation of new knowledge into prevention and treatment of VOD/SOS still need clinical studies. · Reduced intensity myeloablative regimens have proved efficient in reducing the incidence of VOD/SOS.

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