Hemophagocytic syndrome with acute kidney injury accompanied by erythrophagocytic macrophages in the tubular lumen

Hemophagocytic syndrome (HPS) is a life-threatening syndrome involving excessive immune activation. It is often accompanied by renal involvement known as acute kidney injury (AKI), which is a poor prognostic factor of HPS. Thus, early diagnosis and treatment are very important. However, it is rarely identified in renal biopsy specimens, and its major manifestation is acute tubular necrosis. We report a rare case of erythrophagocytic macrophage presence in the tubular lumen of a patient with HPS-associated AKI. A kidney biopsy showed acute tubular necrosis, interstitial massive macrophage infiltration, and phagocytic macrophage casts without glomerular change. Some arteriolar vascular smooth muscle cells showed vacuolization because they were positive for α-smooth muscle actin. The patient’s renal function improved after methylprednisolone pulse therapy followed by oral prednisolone after a month. Our case presents a new pathologic pattern of HPS. Careful urinalysis could suggest renal involvement with HPS. Having knowledge of this pathologic pattern of HPS is important to recognize the disease and to treat it appropriately.