title: Autoimmune hemolysis associated with primary biliary cirrhosis responding to ursodeoxycholic acid as sole treatment.
abstract: Coombs' positive autoimmune hemolytic anemia (AIHA) has been rarely described in association with primary biliary cirrhosis (PBC). The previously reported cases have responded to treatment with a combination of corticosteroids and ursodeoxycholic acid (UDCA). We report a case of AIHA occurring in association with PBC, which has responded to treatment with UDCA alone. Possible mechanisms of autoimmune hemolysis in this patient include bile salt induced immune dysregulation and direct damage to red cell membranes by bile salts leading to exposure of neoantigens and development of red cell autoantibodies. A trial of UDCA as a single agent should be considered as initial treatment in this rare disorder.
authors.affiliation: Sydney University Department of Medicine, Nepean Hospital, Penrith, New South Wales, Australia. sfuller@med.usyd.edu.au
authors.forename: S J
authors.initials: SJ
authors.lastname: Fuller
authors.forename: P
authors.initials: P
authors.lastname: Kumar
authors.forename: M
authors.initials: M
authors.lastname: Weltman
authors.forename: J S
authors.initials: JS
authors.lastname: Wiley
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000209:etiology*; D001323:Autoantibodies / Q000096:biosynthesis; D001324:Autoantigens / Q000276:immunology; D001647:Bile Acids and Salts / Q000097:blood / Q000494:pharmacology; D001853:Bone Marrow / Q000473:pathology; D004910:Erythrocyte Membrane / Q000187:drug effects / Q000276:immunology; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000188:drug therapy; D008565:Membrane Proteins / Q000276:immunology; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D012074:Remission Induction; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D001647:Bile Acids and Salts; D008565:Membrane Proteins; D014580:Ursodeoxycholic Acid