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[Veno-occlusive disease of the liver and POEMS syndrome]
E Andrès1, G Kaltenbach, B Goichot
1Service de Médecine interne, Hôpital de Hautepierre, CHU de Strasbourg.
Background:
Veno-occlusive disease of the liver is a common cause of morbidity and mortality after chemotherapy and/or radiotherapy and bone marrow transplantation. Non thrombotic hepatic venous obstruction is characteristic, but pathogenesis of this disease remains unknown.
Case Report:
In june 1993, a 58-year-old man was treated by cyclophosphamide and steroid for a POEMS syndrome. In november 1996, he developed hepatalgia, edema, ascitis and jaundice. The diagnosis of hepatic veno-occlusive disease was obtained by hepatic biopsy. Refractory ascitis was treated by transjugular intrahepatic portosystemic shunt. Gradual improvement in clinical status was observed after this therapy.
Conclusion:
This observation offers three original types of information: there has been no prior report of an association between veno-occlusive disease and POEMS syndrome, the role of low dose cyclophosphamide in hepatic veno-occlusive disease was certain, transjugular intrahepatic portosystemic shunt is an interesting therapy with one years remission.