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

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.

METHODS

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.

CONCLUSIONS

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.