Association of primary biliary cirrhosis with idiopathic thrombocytopenic purpura |
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Authors: | Toshikuni Nobuyuki Yamato Ryumei Kobashi Haruhiko Nishino Ken Inada Nobu Sakanoue Ritsuko Suehiro Mitsuhiko Fujimura Yoshinori Yamada Gotaro |
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Affiliation: | 1. Department of Internal Medicine,Center for Liver Diseases, Kawasaki Hospital, Kawasaki Medical School, Okayama 7008505, Japan 2. Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine,Dentistry, and Pharmaceutical Sciences, Okayama 7008558, Japan |
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Abstract: | Although both primary biliary cirrhosis (PBC) and idiopathic thrombocytopenic purpura (ITP) are autoimmune diseases, the association of the 2 diseases is rare. Here, we report a case of ITP that developed during the follow-up of PBC in a 74-yearold man. The patient had been diagnosed with PBC 12 years previously, and had received treatment with ursodeoxycholic acid. The platelet count decreased from approximately 60 × 109/L to 8 × 109/L, and the association of decompensated liver cirrhosis (PBC) with ITP was diagnosed. Steroid and immune gamma globulin therapy were successful in increasing the platelet count.Interestingly, human leukocyte antigen genotyping detected the alleles DQBI*0601 and DRBI*0803, which are related to both PBC and ITP in Japanese patients.This case suggests common immunogenetic factors might be involved in the development of PBC and ITP. |
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Keywords: | Primary biliary cirrhosis Idiopathic thrombocytopenic purpura Anti-platelet autoantibody Platelet surface glycoprotein complex Human leukocyte antigen |
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