Immunodeficiency with lymphoid hyperplasia

A 2 1/2-year-old boy presented with recurrent respiratory tract infections, generalized lymphadenopathy and hepatosplenomegaly. Immunologic evaluation revealed a deficiency of humoral immunity. Repeated lymph node biopsies during a 10-year follow-up constantly showed excessive follicular hyperplasia with huge germinal centers consisting of germinoblasts, but lacking plasma cells. The disease can be interpreted as a subtype of the common variable immunodeficiency in which the commutation of germinoblasts to plasma cells in the evolution of B cells is blocked.