A mild, self-resolving case of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis.
- 2022
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. Copyright © 2022 The Authors.
English
2214-2509
10.1016/j.idcr.2022.e01616 [doi] PMC9472058 [pmc] S2214-2509(22)00244-X [pii]
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MedStar Franklin Square Medical Center
MedStar Union Memorial Hospital
Hospitalist
Internal Medicine Residency
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