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Microangiopathies thrombotiques

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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

La microangiopathie thrombotique (MAT) est une entité histologique avec une traduction biologique caractéristique et commune à plusieurs syndromes cliniques. Le syndrome de MAT est dû à la constitution rapide d’une thrombose des petites artères qui entraîne une ischémie viscérale polymorphe, une thrombopénie de consommation et une anémie hémolytique mécanique. Les présentations neurologique ou rénale déterminent respectivement les entités cliniques de purpura thrombotique thrombocytopénique (PTT) et de syndrome hémolytique et urémique (SHU). Cependant, une bicytopénie associant une anémie hémolytique mécanique et une thrombopénie est suffisante pour évoquer le diagnostic de MAT indépendamment des atteintes viscérales associées. Il s’agit de pathologies rares avec une incidence annuelle estimée entre 2 et 6 cas par million d’habitants [1]. La mortalité spontanée des MAT, et en particulier du PTT, est élevée de l’ordre de 90 % avec une évolution imprévisible qui justifie une prise en charge urgente quelle que soit la présentation clinique initiale aussi rassurante soit-elle. L’apport de plasma exogène représente la pierre angulaire du traitement du PTT et de certains SHU et a permis de transformer le pronostic de la pathologie qui est maintenant favorable dans l’immense majorité des cas. Ces vingt dernières années ont vu des avancées décisives dans la compréhension de la physiopathologie des MAT, ce qui a permis d’individualiser différentes entités physiopathologiques avec des perspectives thérapeutiques individualisées [2, 3].

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Authors and Affiliations

  1. Service de réanimation médicale, Hôpital Cochin, AP-HP, 75014, Paris, France

    D. Grimaldi & F. Pène

  2. Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, France

    D. Grimaldi & F. Pène

  3. Unité de médecine transfusionnelle, Hôpital Cochin, AP-HP, 75014, Paris, France

    F. Heshmati

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  1. D. Grimaldi
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Grimaldi, D., Heshmati, F., Pène, F. (2013). Microangiopathies thrombotiques. 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_20

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  • DOI: https://doi.org/10.1007/978-2-8178-0350-0_20

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