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A description about an immunocompetent 61-year old woman who was negative for human immunodeficiency virus and who had recurrent human herpesvirus 8 (HHV-8) infection associated with a relapsing systematic inflammatory syndrome characterized by fever, lymphadenopathy, splenomegaly, edema and rash is presented. The HHV-8 infection, also known as Kaposi's sarcoma (KS)- associated herpesvirus developed 10 months after the initial clinical presentation.

A 27-year old man with the autoimmune lymphoproliferative syndrome and a large-B-cell lymphoma had heterozygous mutations in the Fas and perforin (Prf1) genes where the Fas mutation was inherited from his healthy father and the Prf1 mutation from his healthy mother. The combined effect of the two mutant genes may have contributed to the development of the autoimmune lymphoproliferative syndrome and lymphoma in this patient.