[Veno-occlusive disease of the liver and POEMS syndrome]

Abstract

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.