Sustained virologic response following HCV eradication in two brothers with X-linked agammaglobulinaemia.

X-linked agammaglobulinaemia (XLA) is a humoral immunodeficiency syndrome characterized from childhood by the absence of circulating B lymphocytes, absent or reduced levels of serum immunoglobulin and recurrent bacterial infections. For many affected patients, regular treatment with immunoglobulin is life saving. Hepatitis C viral (HCV) infection acquired through contaminated blood products is widely described in this patient cohort. The natural history of HCV infection in patients with XLA tends to follow a more rapid and aggressive course compared to immunocompetent individuals. Furthermore, standard anti-viral therapy appears to be less efficacious in this patient cohort. Here we report the cases of two brothers with XLA who contracted HCV through contaminated blood products. They were treated with a six month course of Interferon alpha-2b and Ribavirin. We report a sustained virologic response five years after completing treatment.

[1]  M. Gershwin,et al.  Current Perspectives on Primary Immunodeficiency Diseases , 2006, Clinical & developmental immunology.

[2]  H. Chapel,et al.  European surveillance of immunoglobulin safety--results of initial survey of 1243 patients with primary immunodeficiencies in 16 countries. , 2002, Clinical immunology.

[3]  C. Cunningham-Rundles,et al.  Outcome of intravenous immunoglobulin-transmitted hepatitis C virus infection in primary immunodeficiency. , 2001, Clinical immunology.

[4]  S. Frøland,et al.  Long-term outcome of chronic hepatitis C virus infection in primary hypogammaglobulinaemia. , 1999, QJM : monthly journal of the Association of Physicians.

[5]  C. Tiribelli,et al.  Clinical course and risk factors of hepatitis C virus related liver disease in the general population: report from the Dionysos study , 1999, Gut.

[6]  D. Häussinger,et al.  Prognosis of chronic hepatitis c: Results of a large, prospective cohort study , 1998, Hepatology.

[7]  R. Chapman,et al.  Clinical outcome of hypogammaglobulinaemic patients following outbreak of acute hepatitis C: 2 year follow up , 1997, Clinical and experimental immunology.

[8]  G. Rossi,et al.  Outbreak of hepatitis C virus infection in patients with hematologic disorders treated with intravenous immunoglobulins: different prognosis according to the immune status. , 1997, Blood.

[9]  J. Bresee,et al.  Clinical and virologic features of hepatitis C virus infection associated with intravenous immunoglobulin. , 1996, Pediatrics.

[10]  R. Wood.  HEPATITIS C INFECTION IN PATIENTS WITH PRIMARY HYPOGAMMAGLOBULINEMIA AFTER TREATMENT WITH CONTAMINATED IMMUNE GLOBULIN , 1996, Pediatrics.

[11]  A. Giovannetti,et al.  HCV infection in patients with primary defects of immunoglobulin production , 1995, Clinical and experimental immunology.

[12]  A. Alberti,et al.  Hepatitis C viraemia and liver disease in symptom-free individuals with anti-HCV , 1992, The Lancet.

[13]  J. Farrant,et al.  Accessory and T cell defects in acquired and inherited hypogammaglobulinaemia. , 1989, Clinical and experimental immunology.

[14]  M. Doran,et al.  ALPHA-INTERFERON THERAPY FOR NON-A, NON-B HEPATITIS TRANSMITTED BY GAMMAGLOBULIN REPLACEMENT THERAPY , 1987, The Lancet.

[15]  M. Conley.  B cells in patients with X-linked agammaglobulinemia. , 1985, Journal of immunology.

[16]  C. Cunningham-Rundles,et al.  Intravenous immunoglobulin prophylaxis causing liver damage in 16 of 77 patients with hypogammaglobulinemia or IgG subclass deficiency. , 1988, The American journal of medicine.