Haemophagocytic syndrome and HIV

Haemophagocytic syndrome (HPS) is a relatively rare complication of HIV infection but one that can take a particularly aggressive and potentially fatal course. The condition may be underrecognized due to the nonspecific nature of the clinical features. This review summarizes the pathogenesis, clinical and diagnostic features, common associations and current options in the management of HPS in the context of HIV infection, with the aim of increasing diagnostic awareness and facilitating early intervention.

Our understanding of HPS is being increasingly informed by research into the molecular basis of the familial forms of the condition. The list of associated infections and neoplasms continues to lengthen, although the occurrence of HPS is increasingly recognized in the context of HIV infection alone, occasionally as its initial presentation in acute seroconversion. Diagnostic guidelines have recently been refined and attempts to adapt these to particular patient groups are ongoing. Management of HIV-associated HPS is adapted to the clinical context and may involve treatment of an underlying disorder or immunosuppressive therapy to reduce the activation of macrophages and T cells.

The spectrum of HIV-related lymphoproliferative disorders continues to expand. Our understanding of their molecular and cellular basis and the role of infectious organisms in their development increasingly shapes and advances our clinical management of these complex conditions.