Y. Mizukami et al., PRIMARY BILIARY-CIRRHOSIS ASSOCIATED WITH IDIOPATHIC THROMBOCYTOPENICPURPURA, Journal of gastroenterology, 31(2), 1996, pp. 284-288
A case of primary biliary cirrhosis (PBC) associated with idiopathic t
hrombocytopenic purpura (ITP) is reported. The patient is a 59-year-ol
d man. When he was 49 years old, he was diagnosed with ITP and receive
d steroid therapy that successfully increased platelet numbers. Howeve
r, the steroid therapy failed to normalize the elevated gamma-glutamyl
transpeptidase. Ten years after this episode, he suffered from genera
l itching and malaise and exhibited a gradual increase of serum biliar
y enzyme levels. Immunologically, IgM was increased and anti-mitochond
rial antibody was positive. Histological findings of liver needle biop
sy showed chronic non-suppurative destructive cholangitis, confirming
the diagnosis of PBC. To date, very few PBC cases associated with ITP
have been reported. Our case is the second one in Japan. PBC and ITP i
n our patient seemed to develop simultaneously, but the effect of ster
oid therapy on the two conditions was different. This result suggests
that the autoimmune process may have been different in PBC and ITP in
the present patient.