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Ce qu’il faut savoir sur le syndrome d’activation macrophagique en soins intensifs

What we should know about hemophagocytic syndrom in the intensive care unit

  • Session Thématique
  • Published:
Réanimation

Résumé

Le syndrome d’activation macrophagique est une pathologie immunologique rare et potentiellement létale. L’absence de régulation de la réponse lymphocytaire T et NK cytotoxique est responsable d’un état hyperinflammatoire. Il est caractérisé par de la fièvre, une hépatosplénomégalie, des cytopénies, et évolue vers des défaillances d’organe et un état de choc. Une hyperferritinémie, une hypertriglycéridémie et une hypofibrinémie sont souvent observées. Le réanimateur doit savoir le reconnaître pour proposer rapidement un traitement symptomatique spécifique. L’enquête étiologique doit être agressive car seul le traitement de la cause limitera la morbimortalité. Nous discuterons ici la présentation clinique, les modalités du diagnostic, les procédures pour un diagnostic étiologique rapide et les traitements des syndromes d’activation macrophagique de l’adulte à l’exclusion des formes génétiques.

Abstract

Hemophagocytic syndrome (HS) is a rare lifethreatening immunological disorder. Unregulated cytotoxic T and NK lymphocytic response induce a hyperinflammatory state. HS is characterized by fever, hepatomegaly or splenomegaly, cytopenias and may be responsible for multiple organ failure and shock. Hyperferritinemia, hypertriglyceridemia, and hypofibrinemia are commonly seen. Intensive care physicians should be aware of HS to promptly offer an adequate treatment. HS diagnostic evaluation should be aggressive as the treatment of the underlying cause is the key to prevent morbidity and mortality. In this review, we will discuss HS clinical signs, diagnostic criteria, etiologic diagnostic, process and treatments in adults, excluding genetic forms.

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  1. Service d’immunopathologie clinique, hôpital Saint Louis, 1 avenue Claude Vellefaux, F-75010, Paris, France

    L. Galicier

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  1. L. Galicier
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Correspondence to L. Galicier.

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Cet article correspond à la conférence faite par l’auteur au congrès de la SRLF 2014 dans la session: Ces maladies qu’il faut connaitre

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Galicier, L. Ce qu’il faut savoir sur le syndrome d’activation macrophagique en soins intensifs. Réanimation 23 (Suppl 2), 482–490 (2014). https://doi.org/10.1007/s13546-013-0816-2

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  • DOI: https://doi.org/10.1007/s13546-013-0816-2

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