Fulminant hepatic venous thrombosis (Budd-Chiari syndrome) in paroxysmal nocturnal hemoglobinuria: definition of a medical emergency.
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).
D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D004630:Emergencies; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D014859:Warfarin / Q000627:therapeutic use
D014859:Warfarin; D006493:Heparin