Syndrome d'Activation Macrophagique secondaire à un infection à CMV chez un nouveau-né

Abstract

Macrophage activation syndrome is a rare and potentially fatal systemic immunologic disorder. It generally occurs in patients who encounters specific pathogens in the context of preexisting congenital or acquired immune deficiencies. It is associated with a reported 20-30% mortality rates with a much better prognosis in secondary disease. The earlier recognition of its clinical features and treatment are critical. In our patient, immunological workup showed no indication of a familial HLH. Genetic analysis for homozygocy showed that all known genes for FHLH or secondary HLH to metabolic diseases were heterozygous. X-linked forms were excluded. We concluded that CMV was probably the causative agent of secondary macrophage activation syndrome. Further follow-up comforts this idea