[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.
D001778:Blood Coagulation Disorders / Q000209:etiology*; D002648:Child; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D008206:Lymphatic Diseases / Q000150:complications*; D008297:Male; D012480:Salmonella Infections / Q000150:complications*; D012486:Salmonella typhimurium; D018805:Sepsis / Q000150:complications*
Histiocytose réactionnelle systémique avec hémophagocytose et troubles de l'hémostase associés à une granulomatose septique.