ABSTRACT
It is also known as hemophagocyticc syndrome, defined as pathology group characterized in common by the proliferation and the accumulation of macrophages in the bone marrow and the lymphoid system, leading to an abnormal phagocytosis of the blood cells and the production of proinflammatory cytokines. It has a non specific clinical picture as well as a perturbed laboratory findings. We differentiate the hereditary primary forms and the secondary related to several etiologies. Its pathophysiology is not yet clear the treatment remains poorly codified. It is a serious like-threatening disease but recent advances in the comprehension of its physiopathologic mechanisms will allow for a better adapted treatment and improve the survival rate of these patients