PMID: 11910874Mar 26, 2002Paper

Fatal bleeding complications caused by Evans syndrome (autoimmune thrombocytopenia and hemolytic anemia) and type II autoimmune hepatitis in a 56-year-old patient

Medizinische Klinik
Gerhard ZugmaierHans Peter Dienes

Abstract

Autoimmune hepatitis is a rare form of hepatitis of nonviral origin. Two main subentities have been described. The classical lupoid hepatitis (type I) is characterized by hypergammaglobulinemia and the presence of lupus erythematosus cells due to antinuclear antibodies. Autoimmune hepatitis type II, which is associated with antiliver/kidney microsomal antibodies type 1 (LKM 1) shows a more aggressive clinical course than autoimmune hepatitis type I and is frequently (41% of cases) associated with other immunologic diseases. In the present study we report a case of autoimmune hepatitis Type II, associated with autoimmune thrombocytopenia and hemolytic anemia, in a 56-year-old patient. The patient's death was caused by a fatal association of a failing coagulation system due to liver dysfunction and a severe autoimmune thrombocytopenia. The aggressive course of the thrombocytopenia even after splenectomy demonstrated that the splenic enlargement due to the portal hypertension was only a minor factor in the destruction of the thrombocytes. Interestingly, some findings of this case such as the advanced age, the presence of anti-smooth muscle antibodies and HLA-DR4 are usually associated with autoimmune hepatitis type I. The findings...Continue Reading

Citations

Jun 10, 2006·Liver International : Official Journal of the International Association for the Study of the Liver·Edgardo S SantosArie Regev
Jan 8, 2009·Pediatric Blood & Cancer·Paul T JubinskyMary K Short

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