The case of a 45-year-old man with HIV disease with bulky lymphadenopathy, fevers, and weight loss is presented. Immune reconstitution inflammatory syndrome was initially believed to be the cause, but the patient was found to suffer from Kaposi sarcoma, multicentric Castleman disease, hemophagocytic syndrome, and a newly described lymphoproliferative disorder. The diagnostic reasoning related to the causes of the fulminant illness is discussed as well as the roles of Epstein-Barr virus, human herpesvirus 8, and hepatitis C virus in the patient's clinical presentation and the therapeutic choices.
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