Hypogammaglobulinemia

Hypogammaglobulinemia can be primary (congenital) or secondary. It can present in childhood and in adults, and can affect both sexes.

Primary hypogammaglobulinemia may have a delay of several years between clinical presentation and diagnosis. The most common cause is common variable immunodeficiency.

Causes of secondary hypogammaglobulinemia include gastrointestinal losses (e.g., malabsorption/protein-losing enteropathy), nephrotic syndrome, hematologic malignancy, and medication (e.g., immunosuppressives, such as corticosteroids and chemotherapy).

Patients can be predisposed to recurrent upper and lower respiratory tract infections.

Treatment is tailored to specific causes and the clinical presentation of each patient, and includes intravenous or subcutaneous immunoglobulin replacement.

Severe combined immunodeficiency is a medical emergency and suspected cases require immediate specialist center referral for diagnosis and treatment.

Hypogammaglobulinemia is defined as reduced serum immunoglobulin (antibody) levels. This can be due to a variety of underlying primary/congenital intrinsic immune system defects (e.g., common variable immunodeficiency) or secondary immunodeficient states (e.g., medication-related, hematologic malignancy, protein-losing diseases). The most commonly recognized clinical feature is recurrent infection.