Hemophagocytic lymphohistiocytosis (HLH) is a rare, severe, and often fatal disorder. Its hereditary and sporadic form can present as a significant diagnostic challenge to the otolaryngologist. This report describes two fatal cases of adult patients with HLH initially presented as infectious mononucleosis to an otorhinolaryngologist. The clinical presentation, serological and histological features, and management are discussed.
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