Hypogammaglobulinemia

Excerpt

Hypogammaglobulinemia is a disorder caused by low serum immunoglobulin or antibody levels. Immunoglobulins are the main components of the humoral immune system and are able to recognize antigens to trigger a biological response and eradicate the infectious source. Hypogammaglobulinemia is the most common primary immunodeficiency and encompasses a majority of immune-compromised patients. It can be diagnosed in childhood or adulthood. Onset is usually around the second to the third decade in life; however clinical signs of primary hypogammaglobulinemia can occur at any age. Bimodal distribution has been noted in the past with frequent diagnoses between the ages of 6 and 10 and ages 20 to the 40s. Clinical manifestations are rare but have been reported in patients above 50 years old.

This condition predisposes children and adults to recurrent infections, allergies, neoplasms, and autoimmunity. Previous clinical findings suggested these disorders develop in childhood, however, this is now increasingly seen in adult medicine. Common variable immunodeficiency (CVID) is often the cause of hypogammaglobulinemia in adults and X-linked agammaglobulinemia (XLA) is the most common in the pediatric population.