Cutaneous lesions of hemophagocytic syndrome in a patient with T-cell lymphoma and active Epstein-Barr infection.

The hemophagocytic syndrome (HPS) was first described in immune-compromised patients with secondary viral infections. Immunodeficiency has not always been diagnosed before the onset of HPS, but most patients who develop HPS have known immune deficits. HPS has also been reported in association with lymphoreticular malignancies. In HPS the most prominent histologic change is phagocytosis of red blood cells and other bone marrow-derived elements by cytologically benign histiocytes. Considerable confusion exists about the similarities and differences between HPS and other conditions in which hemophagocytosis may occur, for example, histiocytic medullary reticulosis, T-gamma lymphoma, malignant histiocytic lymphoma, and cytophagic histiocytic panniculitis. We report a patient with HPS and cutaneous lesions of cytophagic histiocytic panniculitis who also had an active Epstein-Barr virus infection, and a CD8+ T-cell lymphoma.