Long lasting lymphadenopathy in childhood as an expression of a severe hyperimmune B lymphocyte disorder

On the basis of the 6 cases reported here and scattered published cases, the existence of a childhood chronic immunoblastic lymphadenopathy syndrome is proposed. It is characterized by the following features: A systemic immunoblastic proliferation with varying degrees of maturation resembling the B lymphocyte hyperimmune disorder observed in angioimmunoblastic lymphadenopathy but with no deposits of interstitial amorphous material or vascular proliferation. Disseminated superficial and deep lymphadenopathy. Chronic, pronounced splenomegaly. A constant thrombocytopenia. A polyclonal hypergammaglobulinemia, with markedly elevated antibody titers to various agents. An early onset and a course of several years (up to 20), interrupted in half the cases by the occurrence of a virus-associated (EBV, Papova) neoplastic process or a fatal viral infection. Low natural killer (NK) cell activity in 2 cases.