Bilateral sino-nasal-orbital fungal necrosis associated with hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition caused by hyperactive T cells and macrophages, which release pro-inflammatory cytokines. The diagnosis is based on eight criteria: fever, bi- or tri-lineage cytopenias, splenomegaly, hyperferritinemia, hypofibrinogenemia or hypertriglyceridemia, elevated soluble CD25 levels, low or absent NK cell cytotoxicity, and hemophagocytosis. We report a 1-month-old male infant who was being treated in the pediatric intensive care unit for neonatal infection when he developed six of the eight criteria for HLH, along with bilateral upper eyelid edema that progressed to bilateral sino-nasal-orbital necrosis. MRI revealed a large area of contrast loss involving the nose and both orbits, consistent with fungal necrosis. Biopsy and culture identified <i>Aspergillus fumigatus</i>. Despite antifungal and immunosuppressive treatment, the infant died one week later. We emphasize the need to consider opportunistic fungal infections in HLH patients, particularly when there is rapid and severe clinical deterioration.