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Lancet. 2014 Apr 26;383(9927):1503-1516. doi: 10.1016/S0140-6736(13)61048-X. Epub 2013 Nov 27.

Adult haemophagocytic syndrome.

Author information

1
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.
2
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.
3
Lupus Research Unit, The Rayne Institute, St Thomas' Hospital, King's College University, London, UK.
4
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.

Erratum in

  • Lancet. 2014 Apr 26;383(9927):1464.

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.

PMID:
24290661
DOI:
10.1016/S0140-6736(13)61048-X
[Indexed for MEDLINE]

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