Hemophagocytic lymphohistiocytosis differential diagnosis

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

The differential diagnosis of familial hemophagocytic lymphohistiocytosis includes

• Secondary HLH
• Macrophage-activation syndrome
• Other primary immunodeficiencies that present with hemophagocytic lymphohistiocytosis, such as X-linked lymphoproliferative disease.

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