T-CELL DEFICIENCY IN AN AFRICAN FAMILY

Abstract

We report the case of a 19-month old African boy born from parents with clinical and immunological features suggesting prodromal acquired immunodeficiency syndrome (AIDS). He showed early failure to thrive, generalized lymphadenopathy, bilateral parotitis. Later he developped persistent interstitial pneumonia, persistent oral thrush and mild hepatosplenomegaly. Immunological studies showed hyper IgG level (3.2 g/dl), reversed T-helper/T-suppressor (T4/T8) ratio (19/68) and T-cell defect (cutaneous anergy for different antigens, depressed in vitro response for concanavalin, phytohaemagglutinin and Pokeweed mitogens). Open lung biopsy showed interstitial nodular lymphocytic infiltrates on optic microscopy and cytoplasmic particles on electron microscopy which are of uncertain origin. Recently the two older brothers were investigated. The oldest (6 years) showed chronic parotitis, hyper IgG level (4.7 g/dl), reversed T4/T8 ratio (18/49) and depressed in vitro response to mitogens. The second (5 years) showed slight parotitis, reversed T4/T8 ratio (29/32) and depressed in vitro response to mitogens. Both had normal chest X-rays The clinical and immunological presentation of the three children resemble that of infants with AIDS-like syndrome described by others in the USA. We suggest specific modes of transmission could be involved in familial acquiring of AIDS (transplacental-route, breast feeding, or other routine close-contact).