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A mild, self-resolving case of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disease caused by an excessive activation of the immune system. In most instances, HLH can be fatal without treatment; a life-threatening syndrome driven by a dysregulated immune system and activation of macrophages resulting in cytokine rele...

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Detalles Bibliográficos
Autores principales: Adhikari, Biplov, Rao, Shiavax J., Haas, Christopher J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9472058/
https://www.ncbi.nlm.nih.gov/pubmed/36119758
http://dx.doi.org/10.1016/j.idcr.2022.e01616
Descripción
Sumario:Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disease caused by an excessive activation of the immune system. In most instances, HLH can be fatal without treatment; a life-threatening syndrome driven by a dysregulated immune system and activation of macrophages resulting in cytokine release and consequent cellular damage. HLH can occur as a consequence of multiple genetic abnormalities or environmental triggers. We present an interesting case of mild, self-resolving, HLH due to Epstein-Barr Virus (EBV) infection in a young woman. The best-known diagnostic criteria are based on the HLH-2004 trial, incorporating either the presence of known mutations or five of eight clinical and laboratory findings. Prompt initiation of etoposide-containing therapy is associated with improved survival. Rituximab, an anti-CD20 antibody, can also remove EBV-harboring B-cells and improve outcomes. In a rare subset of patients, the disease can spontaneously resolve without any therapeutic interventions thus sparing the patients from toxic therapies.