Immunoglobulin Deficiencies: The B-Lymphocyte Side of DiGeorge Syndrome

Abstract

DiGeorge syndrome is associated with a T-lymphocyte immunodeficiency. The prevalence of hypogammaglobulinemia has not been reported.

We found that 3% of patients with DiGeorge syndrome were receiving immunoglobulin replacement therapy and 6% of patients over the age of 3 years had hypogammaglobulinemia. We conclude that DiGeorge syndrome is associated with significant humoral immune deficiency.

Keywords

• 22q11.2 deletion syndrome
• syndrome velocardiofacial syndrome
• t-cell production