title: Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years.
abstract: Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of uncontrolled immune activation that has gained increasing attention over the past decade. Although classically known as a familial disorder of children caused by mutations that affect cytotoxic T-cell function, an acquired form of HLH in adults is now widely recognized. This is often seen in the setting of malignancy, infection or rheumatological disorders. We performed a retrospective review across 3 tertiary care centres and identified 68 adults with HLH. The average age was 53 years (range 18-77 years) and 43 were male (63%). Underlying disorders included malignancy in 33 patients (49%), infection in 22 (33%), autoimmune disease in 19 (28%) and idiopathic HLH in 15 (22%). Patients were treated with disease-specific therapy and immunomodulatory agents. After a median follow-up of 32·2 months, 46 patients had died (69%). The median overall survival was 4 months (95% CI: 0·0-10·2 months). Patients with malignancy had a worse prognosis compared to those without (median survival 2·8 months versus 10·7 months, P = 0·007). HLH is a devastating disorder with a high mortality. Further research is needed to improve treatment and outcomes.
authors.affiliation: Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Alison M
authors.initials: AM
authors.lastname: Schram
authors.affiliation: Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors.forename: Paige
authors.initials: P
authors.lastname: Comstock
authors.affiliation: Division of Hematology, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors.forename: Meghan
authors.initials: M
authors.lastname: Campo
authors.affiliation: Department of Radiation Oncology, New York University School of Medicine, New York, NY, USA.
authors.forename: Daniel
authors.initials: D
authors.lastname: Gorovets
authors.affiliation: Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Ann
authors.initials: A
authors.lastname: Mullally
authors.affiliation: Division of Hematology/Oncology, New England Hematology Oncology Associates, Vernon Cancer Center, Newton, MA, USA.
authors.forename: Kelly
authors.initials: K
authors.lastname: Bodio
authors.affiliation: Division of Hematology, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors.forename: Jon
authors.initials: J
authors.lastname: Arnason
authors.affiliation: Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Nancy
authors.initials: N
authors.lastname: Berliner
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007239:Infections / Q000150:complications; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000150:complications; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
keywords: ferritin; haemophagocytic lymphohistiocytosis; haemophagocytosis; immune activation; macrophage activation syndrome