Veno-occlusive disease of the liver and POEMS syndrome

BACKGROUND: Veno-occlusive disease of the liver is a common cause of morbid ity and mortality after chemotherapy and/or radiotherapy and bone marrow tr ansplantation. Non thrombotic hepatic venous obstruction is caracteristic, but pathogenesis of this disease remains unknow. CASE REPORT: In june 1993, a 58-year-old man was treated by cyclophosphamid and steroid for a POEMS syndrome. In november 1996, he developped hepatalg ia, edema, ascitis and jaundice. The diagnosis of hepatic veno-occlusive di sease was obtained by hepatic biopsy. Refractory ascitis was treated by tra nsjugular intrahepatic portosystemic shunt. Gradual improvement in clinical status was observed after this therapy. CONCLUSION: This observation offers three original types of information: th ere has been no prior report of an association between veno-occlusive disea se and POEMS syndrome, the role of low dose cyclophosphamid in hepatic veno -occlusive disease was certain, transjugular intrahepatic portosystemic shu nt is an interesting therapy with one years remission.