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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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Detalles Bibliográficos
Autores principales: Shaw, Tiffany Y., Schivo, Michael
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2016
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.
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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
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