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Transient Hypogammaglobulinemia of Infancy

By James Fernandez, MD, PhD, Clinical Assistant Professor of Medicine; Director, Allergy and Clinical Immunology; Cleveland Clinic, Staff, Department of Allergy and Clinical Immunology, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University; Louis Stokes VA Medical Center, Wade Park;

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Transient hypogammaglobulinemia of infancy is a temporary decrease in serum IgG and sometimes IgA and other Ig isotypes to levels below age-appropriate normal values.

Transient hypogammaglobulinemia of infancy is a primary immunodeficiency disorder that involves humoral immunity deficiencies. In this disorder, IgG levels continue to be low after the physiologic fall in maternal IgG at about age 3 to 6 mo. The cause and inheritance patterns are unknown.

The condition rarely leads to significant infections and is not thought to be a true immunodeficiency. The condition is usually asymptomatic. However, a few patients develop sinopulmonary or GI infections, candidiasis, and/or meningitis.

Diagnosis of transient hypogammaglobulinemia is based on low serum Ig levels (at least 2 standard deviations below the mean for age) and tests showing that antibody production in response to vaccine antigens (eg, tetanus, diphtheria) is normal. Thus, this condition can be distinguished from permanent forms of hypogammaglobulinemia, in which specific antibodies to vaccine antigens are not produced.

Patients with recurrent infections can be temporarily treated with prophylactic antibiotics. Immune globulin is usually unnecessary.

This condition may persist for months to a few years but usually resolves.