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Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of immune dysregulation that is often recognized as secondary to an underlying immune activating state, such as malignancy, rheumatologic disorders, and infections. This case highlights an association between HLH and human immunodeficiency virus (HIV) infection. Although HLH is a rare complication of HIV, it presents a difficult challenge for treatment. Without treatment, HLH is invariably fatal, but the consequence of the immunosuppressive treatment regimen in the setting of an underlying opportunistic infection can also have fatal outcomes.

Fever in a Man with HIV: An Unusual Case of an Immune System Gone Wrong