[Systemic reactive histiocytosis with hemophagocytosis and hemostasis disorders associated with septic granulomatosis].

Authors describe a case of hemophagocytic systemic histiocytosis observed in a child suffering from chronic granulomatous disease, associated with septicemia due to Salmonella typhi murium; outcome was favorable. This type of histiocytic proliferation and activation usually induced by an infectious agent and combined with a congenital or acquired immunological disorder leads to severe clinical and hematological consequences which may contribute to a misleading diagnosis of malignant hematological disease. Moreover, systemic histiocytosis is constantly responsible for early blood clotting abnormalities concerning simultaneously coagulation and fibrinolysis; these changes have to be carefully examined before choosing either heparin or substitutive coagulant fractions.