Activated macrophage syndrome in paediatrics: 4 clinical cases

Abstract

Introduction: The macrophage activation syndrome (MAS) is a rare paediatric condition characterized by enhanced activation of the macrophage and T-cell system, with increased liberation of T-cell cytokines. Clinically it resembles a multiorgan failure syndrome of infectious aetiology. There is still some debate about the syndromes nomenclature, hemato-oncologists usually classify this syndrome as a histiocytic disorder, while rheumatologists define it as MAS. The objective of this publication is to describe 4 patients in which the diagnosis of MAS was made in the early stages of the disease.

Clinical Cases: Two patients had juvenile idiopathic artritis (systemic presentation), 1 with probable immunodeficiency, and in the last case, a familial histiocytosis was suspected. Trigger factors were drugs in 2 patients and 2 infections in the remaining cases. 

Conclusions: The relevance of this report is to show that the early diagnosis and aggressive treatment with steroids and cyclosporine can improve the prognosis of this rare syndrome.