Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult

Andreia Sofia Costa, Anusca Paixão, Henrique Santos, Fernando Salvador


Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.


Disseminated Intravascular Coagulation; Epstein-Barr Virus Infections; Immunocompromised Host; Lymphohistiocytosis, Hemophagocytic; Shock, Septic

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