[Macrophage activation syndrome: a diagnostic challenge]

J A Soult Rubio1, V García Bernabeu, M J Sánchez Alvarez

  • 1Unidad de Cuidados Intensivos Pediátricos, Hospital Infantil Universitario Virgen del Rocío, Sevilla, Spain. jasoult@terra.es

Macrophage activation syndrome, or hemophagocytic syndrome, is a rare disease with high morbidity and mortality. It is a disorder of the mononuclear phagocyte system. Two forms have been described; primary or familial hemophagocytic lymphohistiocytosis and secondary or sporadic hemophagocytic syndrome. Diagnosis of macrophage activation syndrome poses a real challenge for the pediatrician and in many cases is only made at autopsy. We describe two patients with hemophagocytic syndrome associated with parvovirus B19 and Epstein-Barr virus infection.

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