Report of a patient with T-cell deficiency and normal B-cell function: A new immunodeficiency disease with response to transfer factor

Abstract

An 11-yr-old boy had recurrent fevers and pulmonary infections since early childhood and, at age 7, had disseminated varicella with bilateral pneumonitis. A female sibling, age 1, died during this period of time with varicella pneumonia. Two years later, an immunological evaluation showed severe deficits in cellular immunity with skin anergy and very low or poor in vitro lymphocyte proliferative responses to mitogens, allogeneic cells and specific antigens. Quantitation of peripheral T-cells by spontaneous rosette formation was also low—40–45% (normal 61%). On the other hand, B-cell immunity seemed to be completely normal. Serum immunoglobulins and the immunoglobulin receptors on peripheral lymphocytes were normal. The patient produced specific antibodies upon antigen challenge (immunization) and after natural infection. Following transfer factor therapy, conversion of skin reactivity and clinical improvement occurred. No changes were seen in in vitro lymphocyte function with transfer factor therapy. Immunologic reconstitution persisted for 6 mo, after which the patient responded again to the administration of transfer factor. Although this patient has several characteristics in common with Nezelof's syndrome, the patient described in this report appears to represent a distinct clinical entity of primary isolated T-cell deficiency and normal B-cell immunity. The normal B-cell immune system, and the clinical and immunological response to transfer factor therapy, differentiates our patient from the syndrome of thymic dysplasia with immunoglobulin synthesis (Nezelof's syndrome).