Cytokine nephropathy in a patient with fatal Epstein–Barr virus-associated hemophagocytic syndrome

Occasionally, patients with acute Epstein–Barr virus (EBV) infection develop hemophagocytic syndrome (HPS). Acute kidney injury (AKI) is considered a strong prognostic factor, but very few data are available about the biopsy-proven renal involvement of EBV-HPS. Here we describe a previously healthy 17-year-old girl with EBV-HPS. Combination therapy failed and renal necropsy was performed. The renal histology showed that intact glomeruli, remarkable interstitial edema and some cellular infiltration, and protein casts. These findings were compatible with cytokine nephropathy as recently advocated. We suggest that hypercytokinemia may play an important role in the pathophysiology in AKI of EBV-HPS.