Immunoblastic lymphadenopathy with early onset in two boys: immunohistochemical study and indication of decreased proportion of circulating T-helper cells.

Two boys, 5 and 18 years old, had had frequent infections from their second month of life and had later suffered from generalized lymphadenopathy, intermittent maculopapular exanthema, anaemia, and weight loss. Histopathological features of lymph nodes were consistent with immunoblastic lymphadenopathy. Immunohistochemistry showed hyperimmune state with numerous T cells and macrophages along with selective polyclonal IgM-producing cell response in one patient, and IgG- and IgM-producing cells in the other. Both had severe deficiency of serum IgA. Classification of T-cell subsets (OKT4+ and OKT8+) in peripheral blood indicated an imbalance with a relative excess of putative suppressor cells (OKT4+/OKT8+ less than 1; normal, 1.7). It is suggested that decreased T-helper cell activity may explain the observed maturational defects in the B-cell system which probably underlie a hyper-reactive state induced by some sort of triggering, e.g. chronic infection. During steroid treatment the general condition improved remarkably and the lymphadenopathy and hepatosplenomegaly decreased, but the T-cell subset proportions and the serum immunoglobulin levels remained unchanged.