A fatal lymphoproliferative disorder with a suppressor T-cell phenotype. Neoplastic, reactive, or both?

We describe the case of a boy who presented with clinical features of lymphadenitis accompanied by a lymphocytic leukemoid reaction of suppressor/cytotoxic T-cell phenotype. Progressive involvement of the bone marrow, liver, and spleen, and subsequently parotid and lacrimal glands, in the disease process, suggested the alternative diagnosis of malignant lymphoma. An initial dramatic response to Prednisolone was not sustained and he died within 8 weeks of the original onset of symptoms. Postmortem examination revealed extensive lymphocytic infiltration in many organs, and a differential diagnosis of lymphoproliferation is considered. The pathogenesis of this disorder is not established; whether it was essentially neoplastic or reactive is unresolved.