Angioimmunoblastic lymphadenopathy in a child with unusual clinical and immunologic features

We report a case of angioimmunoblastic lymphadenopathy in a child followed for 13 years. Unusual features include prolonged course, cold urticaria, nonthrombocytopenic purpura, poor wound healing, transfusion reactions, and possible neurologic involvement with cerebritis and epileptic seizures. The patient's serum contained a monoclonal cryoglobulin, immunoglobulin G, kappa light chain type, that activated the classic complement pathway in vitro and mediated passive transfer of the cold urticaria. The patient responded well to corticosteroids and has been in clinical remission for 8 years without specific treatment. There is immunologic evidence of persistent residual disease activity. This case illustrates the remarkable diversity of clinical and immunologic features and the variable prognosis of this disorder.