Secondary immune-related hemophagocytic lymphohistiocytosis is a rare but life-threatening complication of immune checkpoint inhibitors. HLH-2004 and HLH-1994 guidelines originally developed for primary HLH are the only available guidelines. It has proven to have a good prognosis if diagnosed promptly with discontinuation of immunotherapy and treated with corticosteroid monotherapy.
Keywords: HLH; hematology/oncology; immune checkpoint inhibitors; ipilimumab; nivolumab; pembrolizumab; secondary hemophagocytic lymphohistiocytosis.
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