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Revista Cubana de Pediatría

versão impressa ISSN 0034-7531versão On-line ISSN 1561-3119

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ALONSO CASTILLO, Amaris; CANTERA OCEGUERA, Dolores; HERNANDEZ HERNANDEZ, Vilma  e  SEIGLIE DIAZ, Frances. Macrophage activation syndrome:: a systemic sepsis simulation. Rev Cubana Pediatr [online]. 2009, vol.81, n.4, pp.76-85. ISSN 0034-7531.

Macrophage activation syndrome is a clinical condition potentially fatal produced by a excessive activation and proliferation of well differentiated macrophages and a intense T lymphocytes proliferation with the consequent cytokines release; its etiology is unknown but the possibility of some triggering agents is exposed including infectious agents (bacteria, fungus, parasites and virus), the drug use such as gold salts, methotrexate, sulfasalazine, aspirin, non-steroid anti-inflammatory drugs, etarnercept, and the stem cells autogenous transplant in patients presenting with autoimmune or hemato-oncologic diseases. Diagnosis is based on clinical, laboratory and histological criteria, if it is suspected in time and an early diagnosis is made, there is a good response to steroid use administered by parenteral rout or cytostatic drugs. The main challenge lies in the presentation similarity with a systemic inflammatory response syndrome or multiorgan failure being necessary to take it into account in pediatric practice and in intensive care units in the face of a child presenting with a severe situation of unknown etiology

Palavras-chave : Macrophage activation syndrome; macrophages; juvenile idiopathic arthritis; systemic sepsis.

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