Síndromes hemofagocíticos: la importancia del diagnóstico y tratamiento precoces.

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Anales de pediatria (Barcelona, Spain : 2003), ISSN: 1695-9531, Vol: 89, Issue: 2, Page: 124.e1-124.e8

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Astigarraga, Itziar; Gonzalez-Granado, Luis I; Allende, Luis M; Alsina, Laia
Elsevier BV
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article description
Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a disorder with high mortality, typically recognised at paediatric age. Without proper treatment, HLH can be fatal. The risk of a rapid progression to multi-organ failure and central nervous system involvement leading to long-term sequelae, are the most feared consequences of a diagnostic delay. Therefore, HLH is a medical emergency that paediatricians should be able to identify in a patient with fever and progressive worsening of general condition. The application of the HLH diagnostic criteria, which include clinical and analytical data (as well as a bone marrow aspirate), and the search for a trigger (infectious, oncological, rheumatological, or metabolic). These are decisive for the establishment of a targeted treatment, which aims at neutralising the trigger and reducing the hyper-inflammation. The most relevant data for general paediatricians are presented in this review, including the physiopathology, diagnosis, and treatment of this serious disease.