TY - JOUR T1 - Hemophagocytic lymphohistiocytosis with immunotherapy: brief review and case report JF - Journal for ImmunoTherapy of Cancer JO - J Immunother Cancer DO - 10.1186/s40425-018-0365-3 VL - 6 IS - 1 SP - 49 AU - Masood Sadaat AU - Sekwon Jang Y1 - 2018/12/01 UR - http://jitc.bmj.com/content/6/1/49.abstract N2 - Background Hemophagocytic Lymphohistiocytosis (HLH), a rare but potentially fatal syndrome of immune hyperactivation, may be an under-recognized immune-related adverse event (irAE). Unlike other irAEs, HLH triggered by immune checkpoint blockade is not well described; no particular diagnostic guidelines and treatment regimens exist. The HLH-2004 criteria remain as the common diagnostic guide. For the treatment of HLH, various combinations of chemotherapeutic, immunosuppressive and glucocorticoid agents are used.Case presentation We report a case of HLH in a 58-year-old metastatic melanoma patient who was undergoing immune checkpoint blockade with pembrolizumab, a programmed cell death-1 (PD-1) receptor inhibitor. The patient presented with fever, upper normal sized spleen, anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, reduced NK cell activity and elevated sCD163 levels, fulfilling the Histiocyte Society HLH-2004 diagnostic criteria. Our patient was successfully treated with oral prednisone (1 mg/kilogram/day), suggesting that HLH from immune checkpoint inhibitors may respond to steroids alone.Conclusion Early diagnosis and treatment of HLH are critical to avoid progressive tissue damage, organ failure and possibly death. HLH should be suspected in clinical presentations with fever, cytopenias and hyperinflammatory markers. HLH in the setting of immune checkpoint blockade may be treated with steroids only but further evidence is required.Abbreviations:BiTeBi-specific T cell engagersCAR TChimeric antigen receptor T cellsCRSCytokine release syndromeCTLA-4Cytotoxic T lymphocyte-associated antigen-4HLHHemophagocytic lymphohistiocytosisirAEImmune-related adverse eventMASMacrophage activation syndromePD-1Programmed death-1PDL-1Programmed death ligand-1 ER -