A case of autoimmune hepatitis associated with idiopathic thrombocytopenic purpura developing severe polyclonal hypergammaglobulinemia

Abstract

We report a case of autoimmune hepatitis associated with idiopathic thrombocytopenic purpura (ITP) developing severe polyclonal hypergammaglobulinemia. A 69-year-old woman was admitted to the Sapporo Medical College Hospital, complaining of general malaise and heaviness in the head. On admission, she was found to have severe polyclonal hypergammaglobulinemia (γ-glovbulin 6.0g/dl, IgG 5, 582mg/dl, IgA 740mg/dl, IgM 1, 938mg/dl). Various autoantibodies including anti-single stranded DNA antibodies, antismooth muscle antibodies and anti-sulfatide antibodies were positive with high titer. Biopsy specimens obtained from the liver revealed those of chronic active hepatitis, showing periportal necrosis with lymphocytic and plasma cell infiltrates.
In addition, the decreased platelet counts (30, 000/mm³) and the elevation of titer of platelet associated IgG (PA-IgG; 234.9ng/10⁷ cells) were found. Bone marrow examination showed normocellularity with slightly elevated number of megakaryocytes (125/mm³).
According to these findings, the patient was diagnosed as autoimmune hepatitis associated with ITP. We started treatment by prednisolone (30mg/day), which resulted in dramatic reduction of hypergammaglobulinemia and recovery of thrombocytopenia.