Transient Hypogammaglobulinemia of Infancy

Transient hypogammaglobulinemia of infancy was first described by Gitlin and Janeway in 1956. Transient hypogammaglobulinemia of infancy is a primary immunodeficiency defined as an immunoglobulin deficiency not accompanied by a B cell defect characterized by prolongation of physiological immunoglobulin deficiency. Immunoglobulins may be low in IgA and IgM levels in addition to low IgG levels. It develops after the age of six months as a result of a decrease in IgG antibodies passed to the child through the placenta and insufficient IgG production by the infant. In these cases, IgG levels remain as low as 2 standard deviations in young infants after 6 months of age. Clinically, it can present with very different conditions. It can be completely asymptomatic or present with recurrent infections. Patients with severe disease may be affected by opportunistic infections, atopy or autoimmunity and may have a more complex course, but by definition the disorder must resolve completely. The prognosis of transient hypogammaglobulinemia of infancy depends on the severity of immunodeficiency. In those with symptomatic or mild disease, the prognosis is good and there is no significant morbidity. A small number of patients may present with a condition similar to X-linked agammaglobulinemia and may require lifelong treatment with antimicrobials and intravenous immunoglobulin.