Hemophagocytic lymphohistiocytosis epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

• Familial hemophagocytic lymphohistiocytosis occurs in approximately 2 in 100,000 individuals worldwide.
• Signs and symptoms of familial hemophagocytic lymphohistiocytosis usually become apparent during infancy, although occasionally they appear later in life. They usually occur when the immune system launches an exaggerated response to an infection, but may also occur in the absence of infection.
• Gender distribution is equal.

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