Systemic reactive histiocytosis with hemophagocytosis and hemostasis disorders associated with septic granulomatosis
Benz-Lemoine, E.; Bordigoni, P.; Schaack, J.C.; Briquel, E.; Chiclet, A.M.; Olive, D.
Archives Francaises de Pediatrie 40(3): 179-182
1983
ISSN/ISBN: 0003-9764 PMID: 6349568 Document Number: 210986
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.