macrophage hyperactivation syndrome with hemophagocytosis

ABSTRACT

The hemophagocytic syndrome is a benign systemic histiocytic proliferation which may be caused by several etiologies infections, autoimmune diseases, hemopathy; << solid >> cancers, immunity deficits. The inadequatee activation of the loop << macrophage - lymphocyte >> with hypercytokinemia explain in the large proportion the physiopathological, clinical and biological features. This syndrome includes fever, organomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and clotting disorders. The diagnosis is based on clinical, biological and especially histopathological criteria with tissue and/or medullary histiocytic infiltration. We report two cases of hemophagocytic syndrome in which the etiology had remained undetermined and the evolution had been fatal