“…All those lead to hemophagocytosis in the bone marrow and the reticulo-endothelial system, which explain clinical and biological symptoms, such as cytopenia or splenomegaly. High levels of interferon γ, interleukin-1 and interleukin-6 play a role in the syndrome, explaining fever or hypertriglyceridemia, for example (4,5,10). Two types of HLH were identified: Familial HLH, related to perforin gene mutation, usually noted in infancy or early childhood, representing therefore, 80 % of the cases.…”