[Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult]
- PMID: 30753807
- DOI: 10.20344/amp.9474
[Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult]
Abstract
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.
A linfohistiocitose hemofagocítica é uma entidade clínica rara, agressiva e que pode colocar em risco a vida do doente, caraterizada por uma ativação imune excessiva. É desencadeada por vários estímulos, em que as infeções desempenham um papel importante. O estímulo infecioso mais comum é a infeção viral, especialmente por Epstein-Barr. As alterações da coagulação são comuns na linfohistiocitose hemofagocítica e a coagulação intravascular disseminada pode estar presente nos casos graves. Apresentamos um caso clínico de linfohistiocitose hemofagocítica secundária a infeção por Epstein-Barr, complicada com coagulação intravascular disseminada, que evoluiu favoravelmente apenas com tratamento de suporte e sem necessidade de tratamento específico.
Keywords: Disseminated Intravascular Coagulation; Epstein-Barr Virus Infections; Immunocompromised Host; Lymphohistiocytosis, Hemophagocytic; Shock, Septic.
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