Immunoglobulin deficiency.

Twenty three patients with primary immunoglobulin(Ig) deficiency were seen during the last ten years. Nine had hypogamma globulinemia (hypo-Ig) and the rest, selective Ig deficiency. Most were in pediatric age group. There was preponderance of males with only one female. Clinical symptoms pertaining to gastrointestinal and sinupulmonary infections were most common. Complete absence of B cells was not observed in any patient with hypogammaglobulinemia. They could be typed as physiological in one patient, X-linked immunodeficiency in 2 patients and common variable immunodeficiency in the remaining six. Three patients with selective IgA deficiency were above 20 years of age. Two had only secretory IgA deficiency, confirmed by jejunal fluid examination and the rest had both secretory and serum IgA deficiency. Low IgM was seen in one patient. We see a spectrum of immunoglobulin deficiencies varying from subtle defects like absence of secretory IgA only, to severe depletion of all immunoglobulins. Therapy is still not ideal due to economic reasons.

Immune status in ataxia telangiectasia.

Immune status of 22 patients of ataxia telangiectasia was studied over a period of 8 yr (mean age of patients: 9.5 +/- 3 yr; 9 of 22 were siblings). Low T-cell number was observed in 14 of 19 patients but the response to PHA challenge done in 10 patients was normal and migration inhibition to BCG antigen was positive in 6 of 6 patients. IgM defect was seen in 2 out of 18 patients and serum IgA was deficient in 10 out of 18 patients. Salivary IgA was also absent in these children. Four children had high spontaneous NBT reduction. None of the patients had lymphoma, leukemia or any other malignancy at the time of presentation. Candida killing was normal in all patients. The presenting feature related to the CNS in almost all children and gross infections were not seen.