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Case Reports
. 2017 Feb 24:26:93.
doi: 10.11604/pamj.2017.26.93.6235. eCollection 2017.

[Macrophage activation syndrome complicating family lymphohistiocytosis]

[Article in French]
Yousra El Boussaadni  1 , Noufissa Benajiba  1 , Ahmed Aziz Bousfiha  2 , Fatima Ailal  2
Affiliations
Case Reports

[Macrophage activation syndrome complicating family lymphohistiocytosis]

[Article in French]
Yousra El Boussaadni et al. Pan Afr Med J. .

Abstract
in English, French

Macrophage activation syndrome (MAS) is an anatomoclinic entity due to inappropriate macrophage activation. It is a rare pathology, characterized by clinical signs that are not very specific and by biological elements. Their association must evoke the diagnosis. It can be classified as primary or secondary, its prognosis is still unclear. We report the case of a 3-year and 4-month-old infant admitted to our department with primary MAS in order to remind clinicians the importance of suspecting primary cause in specific situations.

Le syndrome d'activation macrophagique (SAM) est une entité anatomoclinique due à une stimulation inappropriée des macrophages. Il demeure une pathologie rare, caractérisée par des signes cliniques peu spécifiques et des éléments biologiques dont l'association doit faire évoquer le diagnostic. Il peut être d'origine primaire ou secondaire, son pronostic reste encore sombre. Nous rapportons une observation d'un nourrisson de 3 ans et 4 mois ayant été admis dans notre service pour prise en charge d'une SAM primitif afin de rappeler aux praticiens la nécessité de penser à une cause primitive devant des situations particulières.

Keywords: Macrophage activation syndrome; familial lymphohistiocytosis; fever; genetic study.

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Conflict of interest statement

Les auteurs ne déclarent aucun conflit d'intérêt.

References

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