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

MedStar author(s):
Citation: IDCases. 30:e01616, 2022.PMID: 36119758Institution: MedStar Franklin Square Medical Center | MedStar Union Memorial HospitalDepartment: Hospitalist | Internal Medicine ResidencyForm of publication: Journal ArticleMedline article type(s): Case ReportsSubject headings: IN PROCESS -- NOT YET INDEXEDYear: 2022ISSN:
  • 2214-2509
Name of journal: IDCasesAbstract: 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.All authors: Adhikari B, Haas CJ, Rao SJFiscal year: FY2023Digital Object Identifier: Date added to catalog: 2022-10-20
Holdings
Item type Current library Collection Call number Status Date due Barcode
Journal Article MedStar Authors Catalog Article 36119758 Available 36119758

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

Powered by Koha