Association of primary sclerosing cholangitis, thymoma and hypogammaglobulinemia.

A 64-year-old Japanese woman with thymoma has been suffering from diarrhea and increased alkaline phosphatase levels without jaundice. Her serum immunoglobulin levels of IgM and IgG were less than half of the normal levels, with an increase in CD8 (suppressor/cytotoxic) T cell percentage and a decrease in CD4 (helper) T cell percentage, resulting in a lower CD4/CD8 ratio of 0.31. These immunological features are in accordance with those of hypogammaglobulinemia complicating thymoma. Cholangiography and a liver biopsy specimen disclosed the presence of primary sclerosing cholangitis (PSC). PSC has been recognized in various immunodeficiency syndromes and this case shows that thymoma complicated by hypogammaglobulinemia is associated with PSC.

[1]  J. Bucuvalas,et al.  Papillary stenosis and sclerosing cholangitis in an immunodeficient child. , 1989, Gastroenterology.

[2]  R. Miller,et al.  Sclerosing cholangitis in acquired immunodeficiency syndrome. Case reports and review of the literature. , 1988, Scandinavian journal of gastroenterology.

[3]  L. Ferrell,et al.  Sclerosing cholangitis associated with chronic cryptosporidiosis in a child with a congenital immunodeficiency disorder. , 1987, The American journal of gastroenterology.

[4]  S. Sherlock.  THE SYNDROME OF DISAPPEARING INTRAHEPATIC BILE DUCTS , 1987, The Lancet.

[5]  J. Cello,et al.  Papillary stenosis and sclerosing cholangitis in the acquired immunodeficiency syndrome. , 1987, Annals of internal medicine.

[6]  J. DiPalma,et al.  Primary sclerosing cholangitis associated with hyperimmunoglobulin M immunodeficiency (dysgammaglobulinemia). , 1986, Gastroenterology.

[7]  E. Rosenow,et al.  Disorders of the thymus. A review. , 1984, Archives of internal medicine.

[8]  R. Chapman,et al.  Primary sclerosing cholangitis associated with angioimmunoblastic lymphadenopathy , 1983 .

[9]  H. Mandel,et al.  Primary sclerosing cholangitis associated with immunodeficiency. , 1983, American journal of diseases of children.

[10]  E. Rosenow,et al.  Pulmonary manifestations of hypogammaglobulinaemia. , 1978, Thorax.

[11]  R. Wedgwood,et al.  LIVER DISEASE AND IMMUNODEFICENCY SYNDROMES , 1974 .

[12]  A. Eddleston,et al.  Intrahepatic sclerosing cholangitis associated with a familial immunodeficiency syndrome. , 1973, Lancet.

[13]  S. Sherlock,et al.  Primary sclerosing cholangitis, the biliary tree, and ulcerative colitis. , 1967, Gut.

[14]  M. Rubin,et al.  CLINICAL DISORDERS ASSOCIATED WITH THYMIC TUMORS. , 1964, Archives of internal medicine.

[15]  L. Woolner,et al.  Sclerosing cholangitis: its possible association with Riedel's struma and fibrous retroperitonitis. Report of two cases. , 1963, The New England journal of medicine.