Hemophagocytic Syndrome Induced by HIV

Abstract

Hemophagocytic syndrome is an unusual illness due to the proliferation of activated macrophages phagocyting the hematopoietic cells. This syndrome has been reported in association with infectious agents, lymphoproliferative diseases, immunosuppressive conditions or auto-immune illnesses. Cytokines secreted by activated or neoplastic lympho-cytes activate the macrophages. Autocrine and paracrine mechanisms take over and could explain resistance to therapy. Some viruses, including EBV, could upregulate cytokine genes. This report describes an adult with HIV-infection presenting with severe hemophagocytic syndrome in whom an extensive evaluation did not reveal opportunistic disease and suggests that the cause was the HIV. The outcome was fatal and highlights the poor prognosis of hemophagocytic syndrome in this context.

Keywords: AIDS; HIV-1; histiocytosis; non-Langerhans-cell.