Pathophysiology of organ dysfunction in the macrophage activation syndrome

Abstract

Hemophagocytic syndrome is a rare but severe, potentially life-threatening, disease. It is characterized by clinic and biologic features secondary a non regulated cytokines secretion leading to proliferation and activation of benign macrophages with hemophagocytosis. Hemophagocytic syndrome may be secondary to infection, haematological malignancies, connective tissue disease and specific immundeficiency in children. Early diagnosis and therapeutic initiation is mandatory since mortality is as high as 50%. Diagnosis should be evocated in patients with febrile pancytopenia associated with one or several organ failures. Increased plasma ferritine and triglycerides rates help toward the diagnosis. However, only pictures of hemophagocytosis within marrow smears or lymph nodes, marrow, or liver biopsies might confirm the diagnosis, and sometimes help to find the aetiology of the hemophagocytic syndrome. Beyond therapies based on each aetiology, specific hemophagocytic syndrom treatment is required. © 2005 Société de réanimation de langue française. Publié par Elsevier SAS. Tous droits réservés.