title: Adult haemophagocytic syndrome.
abstract: Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage--mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder.
authors.affiliation: Josep Font Laboratory of Autoimmune Diseases-CELLEX, Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.affiliation: Josep Font Laboratory of Autoimmune Diseases-CELLEX, Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Brito-Zerón
authors.affiliation: Department of Haematology, Institut Clínic de Malalties Hematològiques i Oncològiques, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Armando
authors.initials: A
authors.lastname: López-Guillermo
authors.affiliation: Lupus Research Unit, The Rayne Institute, St Thomas' Hospital, King's College University, London, UK.
authors.forename: Munther A
authors.initials: MA
authors.lastname: Khamashta
authors.affiliation: Department of Internal Medicine, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain. Electronic address: xavbosch@clinic.ub.es.
authors.forename: Xavier
authors.initials: X
authors.lastname: Bosch
mesh_terms: D000328:Adult; D014943:Global Health; D006801:Humans; D015994:Incidence; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy