Association of primary biliary cirrhosis with idiopathic thrombocytopenic purpura.
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-year-old 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 x 10(9)/L to 8 x 10(9)/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 DQB1*0601 and DRB1*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.
Toshikuni
Nobuyuki
N
Department of Internal Medicine, Center for Liver Diseases, Kawasaki Hospital, Kawasaki Medical School, 2-1-80 Nakasange, Okayama 7008505, Japan. n.toshikuni@gmail.com
Yamato
Ryumei
R
Kobashi
Haruhiko
H
Nishino
Ken
K
Inada
Nobu
N
Sakanoue
Ritsuko
R
Suehiro
Mitsuhiko
M
Fujimura
Yoshinori
Y
Yamada
Gotaro
G
D000368:Aged; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D006683:HLA-DQ Antigens / Q000235:genetics; D059866:HLA-DQ beta-Chains; D006684:HLA-DR Antigens / Q000235:genetics; D059811:HLA-DRB1 Chains; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D008297:Male; D010976:Platelet Count; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D013256:Steroids / Q000627:therapeutic use; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; C466440:HLA-DRB1*08:01 antigen; D016756:Immunoglobulins, Intravenous; D013256:Steroids; D014580:Ursodeoxycholic Acid