Haemophagocytic syndromes: The importance of early diagnosis and treatment
Por:
Astigarraga I, Gonzalez-Granado LI, Allende LM and Alsina L
Publicada:
1 ago 2018
Ahead of Print:
2 jun 2018
Categoría:
Pediatrics, perinatology and child health
Resumen:
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, ontological, 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. (C) 2018 Asociacion Espanola de Pediatria. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license.
Filiaciones:
Astigarraga I:
Servicio de Pediatría, Biocruces Health Research Institute, Hospital Universitario Cruces, UPV/EHU. Barakaldo, Bizkaia, España
Gonzalez-Granado LI:
Unidad de Inmunodeficiencias, Pediatría, Hospital Universitario 12 de Octubre, Instituto de investigación i+12. Universidad Complutense, Madrid, España
Allende LM:
Servicio de Inmunología, Hospital Universitario 12 de Octubre, Instituto de investigación i+12, Universidad Complutense, Madrid, España
Alsina L:
Unidad de Inmunología Clínica e Inmunodeficiencias Primarias, Sección de Alergia e Inmunología Clínica pediátrica, Hospital Sant Joan de Déu, Institut de Recerca Pediàtrica Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, España
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