title: Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in a rheumatic patient receiving abatacept therapy.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a life- threatening hyperinflammatory disease that causes extensive organ damage. It is generally triggered by viral, fungal, or parasitic infections in the setting of hematologic disease-induced immune deficiency. Occurrences in rheumatologic disease are less frequent, with the syndrome developing most often in patients with systemic lupus erythematosus and adult-onset Still disease. It is believed that the immunosuppression induced by rheumatologic disease itself and exacerbation by immunomodulatory therapies predispose to infection and subsequently HLH. Abatacept is a relatively new disease-modifying agent for rheumatoid arthritis (RA) that has been associated with varicella zoster virus, cytomegalovirus, and Epstein-Barr virus (EBV) infections, but not previously in the setting of HLH. Here we report a unique case of EBV-associated HLH in a RA patient receiving abatacept therapy.
authors.affiliation: Medical Student, Class of 2015, at The Warren Alpert Medical School of Brown University.
authors.forename: Rahul S
authors.initials: RS
authors.lastname: Dalal
authors.affiliation: Assistant Professor of Pathology and Laboratory Medicine, The Warren Alpert Medical School of Brown University.
authors.forename: Mark P
authors.initials: MP
authors.lastname: LeGolvan
authors.affiliation: Assistant Professor of Pathology and Laboratory Medicine, The Warren Alpert Medical School of Brown University.
authors.forename: Dariusz R
authors.initials: DR
authors.lastname: Stachurski
authors.affiliation: Assistant Professor of Medicine, The Warren Alpert Medical School of Brown University.
authors.forename: Laura R
authors.initials: LR
authors.lastname: Goldberg
mesh_terms: D000069594:Abatacept; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001172:Arthritis, Rheumatoid / Q000188:drug therapy*; D020031:Epstein-Barr Virus Infections; D017809:Fatal Outcome; D006801:Humans; D018796:Immunoconjugates / Q000627:therapeutic use*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000821:virology*; D008297:Male; D008875:Middle Aged
chemical_list: D018501:Antirheumatic Agents; D018796:Immunoconjugates; D000069594:Abatacept
keywords: Epstein-Barr virus; Hemophagocytic lymphohistiocytosis; abatacept; rheumatoid arthritis