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Syndrome d’activation lymphohistiocytaire

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Book cover Maladies rares en médecine d’urgence

Part of the book series: Références en médecine d’urgence. Collection de la SFMU ((REFERMED))

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Résumé

Le syndrome d’activation lymphohistiocytaire (SALH) est le fruit d’une réponse lymphocytaire T cytotoxique non régulée. Il aboutit à un tableau de pancytopénie fébrile auquel peuvent rapidement s’ajouter des défaillances d’organe qui en font toute la gravité. Lorsque les défaillances d’organes menacent le pronostic vital du patient, des traitements symptomatiques peuvent être rapidement efficaces. Ceux-ci devront être menés de front avec une enquête étiologique agressive. Seul un traitement étiologique permettra d’éviter Ta résurgence du SALH. La littérature, de plus en plus étendue sur le sujet, rapporte l’occurrence de SALH au cours de nombreuses patnologies compliquant la démarche diagnostique. Toutefois, en pratique courante, un petit nombre de causes expliquent la large majorité des SALH permettant d’arriver rapidement à un diagnostic avec une enquête ciblée en fonction du terrain sous-jacent. Quatre grands groupes d’étiologies sont maintenant clairement identifiés. Les SALH primitifs ou familiaux, liés à une anomalie génétique identifiée ou non, sont diagnostiqués essentiellement en médecine pédiatrique. Les SALH secondaires se rencontrent à tous les âges; ils représentent la très large majorité des SALH de l’adulte. On distingue les SALH secondaires aux hémopathies lymphoïdes, aux agents infectieux et les SALH associés aux maladies systémiques.

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  1. Service d’immunologie clinique, Hôpital Saint-Louis, AP-HP, 75010, Paris, France

    L. Galicier

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Galicier, L. (2013). Syndrome d’activation lymphohistiocytaire. In: Maladies rares en médecine d’urgence. Références en médecine d’urgence. Collection de la SFMU. Springer, Paris. https://doi.org/10.1007/978-2-8178-0350-0_10

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  • DOI: https://doi.org/10.1007/978-2-8178-0350-0_10

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