title: Crohn's disease complicated by Epstein-Barr virus-driven haemophagocytic lymphohistiocytosis successfully treated with rituximab.
abstract: We report a case of Epstein-Barr virus (EBV)-driven haemophagocytic lymphohistiocytosis (HLH) in a man with Crohn's disease treated with 6-mercaptopurine and adalimumab therapy who was successfully treated with rituximab therapy alone. This is the first published case in an adult patient with EBV-driven HLH in the setting of thiopurine use and inflammatory bowel disease to be successfully treated with rituximab therapy alone. Here, we will discuss putative immunological mechanisms which may contribute to this potentially life-threatening complication.
authors.affiliation: Department of Immunology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
authors.forename: Grace
authors.initials: G
authors.lastname: Thompson
authors.affiliation: Department of Haematology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
authors.forename: Dominic
authors.initials: D
authors.lastname: Pepperell
authors.affiliation: Harry Perkins Institute of Medical Research Perkins South, Perth, Bahrain.|Centre for Inflammatory Bowel Diseases, Subiaco, Perth, Western Australia, Australia.
authors.forename: Ian
authors.initials: I
authors.lastname: Lawrence
authors.affiliation: Department of Immunology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
authors.forename: Benjamin David
authors.initials: BD
authors.lastname: McGettigan
mesh_terms: D000068879:Adalimumab / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003424:Crohn Disease / Q000150:complications / Q000188:drug therapy; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000009:adverse effects; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy* / Q000821:virology*; D008297:Male; D015122:Mercaptopurine / Q000009:adverse effects; D000069283:Rituximab / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D015122:Mercaptopurine; D000068879:Adalimumab