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.
D001402:B-Lymphocytes; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D006529:Hepatomegaly / Q000150:complications; D006801:Humans; D006965:Hyperplasia; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008198:Lymph Nodes / Q000473:pathology; D008221:Lymphoid Tissue / Q000473:pathology*; D008297:Male; D013163:Splenomegaly / Q000150:complications; D013601:T-Lymphocytes