Fulminant hepatic venous thrombosis (Budd-Chiari syndrome) in paroxysmal nocturnal hemoglobinuria: definition of a medical emergency

Johns Hopkins Med J. 1980 Jun;146(6):247-54.

Abstract

Hepatic venous thrombosis (HVT) should be recognized as a distinct and highly lethal thrombotic complication of paroxysmal nocturnal hemoglobinuria. In a patient with fulminant onset prompt recognition of a triad of clinical, laboratory and liver scan findings facilitated early, aggressive and prolonged heparinization followed by coumadin maintenance, all with good results. Additionally a case of asymptomatic, smoldering HVT was unearthed by liver scan survey and confirmed by hepatic venogram; the patient was started on a regimen of Coumadin (crystalline sodium warfarin, Endo).

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Budd-Chiari Syndrome / diagnosis
  • Budd-Chiari Syndrome / drug therapy
  • Budd-Chiari Syndrome / etiology*
  • Emergencies
  • Hemoglobinuria, Paroxysmal / complications*
  • Heparin / therapeutic use
  • Humans
  • Male
  • Middle Aged
  • Warfarin / therapeutic use

Substances

  • Warfarin
  • Heparin