PARTIAL RECONSTITUTION OF A PATIENT WITH CONGENITAL THYMIC DYSPLASIA BY FETAL TISSUE AND TRANSFER FACTOR

Abstract

Attempts at immunologic reconstitution of patients with congenital thymic dysplasia have been disappointing. We have studied an 18 month old male with chronic history of mucocutaneous candidiasis (CMC), diarrhea, repeated infections and failure to thrive. He had a marked deficiency of T-cell functions with a decrease in circulating T-cells, absent cutaneous reactivity to skin test antigens and absent PHA responsiveness in vivo and in vitro. Although lymphopenic he had adequate numbers of circulating B-cells and plasma cells in his bone marrow aspirates. B-cell functions including levels of serum immunoglobulins, isohemagglutinins and pokeweed mitogen responsiveness were within the normal range. Implanted tissues from a female abortus of 16 weeks gestation restored in vivo and in vitro PHA responsiveness within 24 hours. Karyotypic and HL-A analyses demonstrated that chimerism had not occurred. In an attempt to augment persistently negative responses to Candida antigen, multiple doses of transfer factor (2.4 × 108 lymphocytes/dose) were administered. Peripheral T-cell rosettes increased from 5% to 40% without change in his response to Candida antigen or his clinical CMC. After eight months of continuing transfer factor therapy the patient has tripled his weight and remains clinically stable.