The autopsy case of primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura

Abstract

This is the first case of primary biliary cirrhosis (PBC) associated with idiopathic thrombocytopenic purpura(ITP)as far as we examined.
The patient:57 year-old female, was admitted to our hospital because of liver dysfunction in 1974. The laboratory data revealed serum total birirubin 1.6mg/dl, Al-P 61.1 K.A.U., moderate elevation of transaminase and marked elevation of IgM. Antimitochondrial antibody (AMA) was positive. In 1975, a gingival and nasal bleeding appeared. The platelet count was 2000. Bone marrow examination revealed normocellular marrow and slight increase of megakaryocytes. Treatment with predonisolone was markedly effective for thrombocytopenia and bleeding tendency. She underwent splenectomy and liver wedge biopsy in 1977. The histological findings showed those of CNSDC, compatible with stage I of PBC (Sheuer). Splenectomy was not effective for thrombocytopenia and she was given predonisolone again. On the basis of clinical course and laboratory findings, diagnosis of ITP was established. She died on Nov. 1983. Autopsy showed stage IV of PBC and tuberculous endometritis.