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Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease caused by excessive immune activation. Acquired HLH is seen in adults and is often caused by infection or malignancy. Diagnosis is difficult and usually missed as clinical and laboratory findings are nonspecific. Moreove...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4855217/ https://www.ncbi.nlm.nih.gov/pubmed/27200381 http://dx.doi.org/10.1177/2324709616647409 |
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author | Shaw, Tiffany Y. Schivo, Michael |
author_facet | Shaw, Tiffany Y. Schivo, Michael |
author_sort | Shaw, Tiffany Y. |
collection | PubMed |
description | Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease caused by excessive immune activation. Acquired HLH is seen in adults and is often caused by infection or malignancy. Diagnosis is difficult and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to HLH. Thus, in patients presenting with presumed severe sepsis or septic shock complicated by multiorgan failure without a clear infectious source, HLH should be considered. A disproportionately high ferritin may be one of the earlier laboratory findings to suggest HLH. We discuss a case of a young male who presented with presumed septic shock with multiorgan failure who was eventually found to have Epstein-Barr virus–induced HLH. |
format | Online Article Text |
id | pubmed-4855217 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-48552172016-05-19 Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis Shaw, Tiffany Y. Schivo, Michael J Investig Med High Impact Case Rep Case Report Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease caused by excessive immune activation. Acquired HLH is seen in adults and is often caused by infection or malignancy. Diagnosis is difficult and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to HLH. Thus, in patients presenting with presumed severe sepsis or septic shock complicated by multiorgan failure without a clear infectious source, HLH should be considered. A disproportionately high ferritin may be one of the earlier laboratory findings to suggest HLH. We discuss a case of a young male who presented with presumed septic shock with multiorgan failure who was eventually found to have Epstein-Barr virus–induced HLH. SAGE Publications 2016-05-02 /pmc/articles/PMC4855217/ /pubmed/27200381 http://dx.doi.org/10.1177/2324709616647409 Text en © 2016 American Federation for Medical Research http://creativecommons.org/licenses/by/3.0/ This article is distributed under the terms of the Creative Commons Attribution 3.0 License (http://www.creativecommons.org/licenses/by/3.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Case Report Shaw, Tiffany Y. Schivo, Michael Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis |
title | Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis |
title_full | Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis |
title_fullStr | Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis |
title_full_unstemmed | Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis |
title_short | Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis |
title_sort | weathering a cytokine storm: a case of ebv-induced hemophagocytic lymphohistiocytosis |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4855217/ https://www.ncbi.nlm.nih.gov/pubmed/27200381 http://dx.doi.org/10.1177/2324709616647409 |
work_keys_str_mv | AT shawtiffanyy weatheringacytokinestormacaseofebvinducedhemophagocyticlymphohistiocytosis AT schivomichael weatheringacytokinestormacaseofebvinducedhemophagocyticlymphohistiocytosis |