We report the case of a 36-year-old patient admitted for Macrophage Activation Syndrome (MAS), revealed by inflammatory dermo-hypodermal lesions, tumor syndrome, pancytopenia, biological cholestasis, increased lactate dehydrogenase rates and hemophagocytosis in skin biopsies. The patient also exhibited an exponential increase in triglycerides serum levels that reached 22 g/L. The patient died after multiple organ failure. This case illustrates the value of the hypertriglyceridemia in the diagnosis and monitoring of hemophagocytic syndrome but also highlights the difficulty in understanding its pathophysiology especially with several elements that contribute to its aggravation during the evolution of this syndrome.
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