Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome characterized by excessive immune activation, and diagnosing it in patients with HIV is challenging due to overlapping clinical and laboratory findings. We present the case of a 69-year-old Afro-Caribbean female with advanced HIV, partial adherence to antiretroviral therapy, and Epstein-Barr virus (EBV) infection, who developed HLH. Despite initial management with antibiotics, antiretroviral therapy, and supportive care, her condition deteriorated with worsening thrombocytopenia, renal and liver impairment, and elevated inflammatory markers. The clinical syndrome was most consistent with HLH, with a calculated HLH probability score of >99%. EBV DNA levels and high interleukin-2 receptor levels confirmed EBV as the trigger for HLH. Treatment with dexamethasone, intravenous immunoglobulin, etoposide, and rituximab was initiated, but the patient developed severe pancytopenia and renal failure, ultimately passing away. This case underscores the need for a heightened index of suspicion for HLH in HIV patients and the complexities of its management, including the limitations of the HLH-94 regimen in immunocompromised individuals. Further research is required to determine the optimal treatment approach for HIV-associated HLH.