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An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis
Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7473604/ https://www.ncbi.nlm.nih.gov/pubmed/32905485 http://dx.doi.org/10.7759/cureus.9561 |
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author | Quadri, Syed P Jain, Nitesh K Brandon, Brooke L Modi, Harshit Bawaadam, Hasnain |
author_facet | Quadri, Syed P Jain, Nitesh K Brandon, Brooke L Modi, Harshit Bawaadam, Hasnain |
author_sort | Quadri, Syed P |
collection | PubMed |
description | Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in a susceptible host resulting in immune activation and dysregulation leading to disruption of immune homeostasis, cytokine storm and multi-organ failure. We describe a case of Epstein-Barr virus (EBV) associated HLH with its typical diagnostic challenges and associated high mortality rate. Certain diagnostic criteria and online tools may help to arrive at an earlier presumptive diagnosis which, in turn, may expedite treatment and lead to better clinical outcomes. |
format | Online Article Text |
id | pubmed-7473604 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-74736042020-09-04 An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Quadri, Syed P Jain, Nitesh K Brandon, Brooke L Modi, Harshit Bawaadam, Hasnain Cureus Infectious Disease Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in a susceptible host resulting in immune activation and dysregulation leading to disruption of immune homeostasis, cytokine storm and multi-organ failure. We describe a case of Epstein-Barr virus (EBV) associated HLH with its typical diagnostic challenges and associated high mortality rate. Certain diagnostic criteria and online tools may help to arrive at an earlier presumptive diagnosis which, in turn, may expedite treatment and lead to better clinical outcomes. Cureus 2020-08-05 /pmc/articles/PMC7473604/ /pubmed/32905485 http://dx.doi.org/10.7759/cureus.9561 Text en Copyright © 2020, Quadri et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Infectious Disease Quadri, Syed P Jain, Nitesh K Brandon, Brooke L Modi, Harshit Bawaadam, Hasnain An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis |
title | An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis |
title_full | An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis |
title_fullStr | An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis |
title_full_unstemmed | An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis |
title_short | An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis |
title_sort | intriguing presentation of epstein-barr virus-associated hemophagocytic lymphohistiocytosis |
topic | Infectious Disease |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7473604/ https://www.ncbi.nlm.nih.gov/pubmed/32905485 http://dx.doi.org/10.7759/cureus.9561 |
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