Cirrhose biliaire primitive et purpura thrombopénique idiopathique. Une association inédite

A case of primary biliary cirrhosis (PBC) associated with idiopathic thrombocytopenic purpura (ITP) is reported. The patient is a 59-year-old man. When he was 49 years old, he was diagnosed with ITP and received steroid therapy that successfully increased platelet numbers. However, the steroid therapy failed to normalize the elevated gamma-glutamyl transpeptidase. Ten years after this episode, he suffered from general itching and malaise and exhibited a gradual increase of serum biliary enzyme levels. Immunologically, IgM was increased and anti-mitochondrial antibody was positive. Histological findings of liver needle biopsy 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 in our patient seemed to develop simultaneously, but the effect of steroid 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.

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Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura

Mizukami

Ohhira

Matsumoto

et al. 1996

J Gastroenterol

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Idiopathic Thrombocytopenic Purpura in a Liver Transplant Recipient with Previous Primary Biliary Cirrhosis

Yoshida

Mandl

Erb

et al. 1997

Journal of Clinical Gastroenterology

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The loss of immunotolerance has been implicated in the pathogenesis of both primary biliary cirrhosis (PBC) and idiopathic, immune-mediated thrombocytopenic purpura (ITP). An association between these two autoimmune diseases has been well described. We describe a 41-year-old woman in whom ITP developed 457 days after liver transplantation for PBC while receiving immunosuppressive medications sufficient to maintain allograft function. Our case report, the first to describe post-transplant ITP in association with PBC, demonstrates the persistence of the underlying immune dysregulation of PBC after transplantation. The practice of decreasing the dosage of immunosuppressive medication to maintenance levels after transplantation may unmask the effects of this defect in immunotolerance.

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Correspondence

1990

Journal of Internal Medicine

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Cirrhose biliaire primitive et purpura thrombopénique idiopathique. Une association inédite

Cited by 3 publications

References 15 publications

Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura

Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura

Idiopathic Thrombocytopenic Purpura in a Liver Transplant Recipient with Previous Primary Biliary Cirrhosis

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