Syndrome d'activation macrophagique d'origine infectieuse : étiologies et prise en charge

Hemophagocytic syndrome (HPS) is a rare and life-threatening disease. It is characterized by clinical criteria (fever, splenomegaly), biological features (cytopenias, low fibrinogen, high plasma triglycerids, and hyperferritinaemia) and pictures of hemophagocytosis within marrow, spleen or lymph nodes, caused by a dysregulation in cytokine secretion and activation and benign proliferation of lymphocytes or histiocytes. Among various aetiologies of HPS (primary genetic deficiency in children, haematological and solid malignancies, connective tissue disease), infection, most frequently by viruses, is an important one. The infectious agent responsible for HPS must be actively searched and promptly treated. Organ failures must be supported in an intensive care unit. An early administration of etoposide seems to be the cornerstone of HPS specific treatment.