Sex-linked agammaglobulinemia (Bruton's disease). A case report.

Abstract

X-linked agammaglobulinemia (Bruton's disease) is a rare disease characterized by marked decrease in all classes of immunoglobulins and absence of circulating B cells and plasma cells. The affected boys frequently present with recurrent respiratory tract infections after 6 months to 2 years of age. A combination regimen of intravenous human immunoglobulins and strong parenteral antibiotics remains the stone of treatment. Herein we report a one year and ten months old boy with Bruton's disease. He has had repeated infections of upper and lower respiratory tract since 6 months of age. Immunological studies revealed panhypogammaglobulinemia, nearly total absence of circulating B cells, negative isohemagglutinins and reversed CD4/CD8 ratio. Pedigree analysis suggests a sex-linked recessive inheritance. Intravenous immunoglobulins as well as parenteral antibiotics were administered to overcome pneumonia and purulent otitis media. He is currently undergoing co-trimethoxazole prophylaxis and periodic intravenous immunoglobulins infusion.

• Authors: J L Chung, J L Huang, I J Hung
• Journal: Changgeng yi xue za zhi (Changgeng Yi Xue Za Zhi) Vol. 15 Issue 1 Pg. 39-43 (Mar 1992) China (Republic : 1949- )
• PMID: 1581837 (Publication Type: Case Reports, Journal Article)

Topics

• Agammaglobulinemia (genetics, immunology, therapy)
• B-Lymphocytes (immunology)
• Genetic Linkage
• Humans
• Infant
• Male
• Sex Chromosomes