Transient hypogammaglobulinemia of infancy: Review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up

Abstract

The clinical and immunologic features of 11 patients with transient hypogammaglobulinemia of infancy are reported and compared with those of the 16 patients previously reported. Seven were re-evaluated three to ten years after infancy. Two groups were identified: six who were found by screening relatives of patients with other types of immunodeficiency and five whose sera were sent because the patients were having frequent or unusual infections. Those in the first group had no significant health problems during early infancy or childhood. In the second group recurrent infection was a problem during early infancy but not in later years; the latter patients also frequently had other health problems. Serum immunoglobulin concentrations were entirely normal at the last evaluation in five of the six THI patients with immunodeficient relatives. In the second group, the concentrations of one or more immunoglobulin classes, although greatly increased, were still below the normal range. All 11 patients were found to be capable of synthesizing antibodies to human type A and B erythrocytes and to diphtheria and tetanus toxoids, usually by 6 to 11 months of age, and well before immunoglobulin concentrations became normal. Lymphocyte studies in vitro showed no abnormalities in the percentages of cells in the different subpopulations or in their responses to the mitogens. None of the patients was given immune serum globulin replacement therapy and none experienced serious infections during their period of follow-up. The finding of only 11 cases of THI among over 10,000 patients whose sera were sent for immunoglobulin studies over a 12-year period suggests that this is not a common entity.