Secondary hemophagocytic lymphohistiocytosis (hemophagocytic syndrome, massive macrophage activation syndrome) is a rare, aggressive, severe syndrome with a frequent fatal outcome. The pathogenesis of the syndrome, the role of viral infection, and the development of an excessive, aberrant, and inflammatory response leading to multiple organ dysfunction and sepsis remain unclear so far. The paper presents the data available in the literature and describes the authors' own observation of secondary hemophagocytic lymphohistiocytosis in a 14-year-old girl who had Epstein-Barr virus infection and died of severe multiple organ dysfunction.
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