Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus...

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Main Authors: Neda Hashemi-Sadraei, Pimprapa Vejpongsa, Muhamed Baljevic, Lei Chen, Modupe Idowu
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2015/491567
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author Neda Hashemi-Sadraei
Pimprapa Vejpongsa
Muhamed Baljevic
Lei Chen
Modupe Idowu
author_facet Neda Hashemi-Sadraei
Pimprapa Vejpongsa
Muhamed Baljevic
Lei Chen
Modupe Idowu
author_sort Neda Hashemi-Sadraei
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. As clinical and laboratory features of HLH could overlap with septic shock syndrome in most patients, the diagnosis of HLH, especially in adults, is the most challenging aspect of the disease that results in delayed recognition and treatment of rapidly progressive multiorgan system failure. We report a case of Hemophagocytic lymphohistiocytosis in a patient who presented with signs of septic shock syndrome and we review the literature on the topic.
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institution Kabale University
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series Case Reports in Hematology
spelling doaj-art-66f6a7cc969448b281dc5d10fcce852f2025-02-03T05:52:45ZengWileyCase Reports in Hematology2090-65602090-65792015-01-01201510.1155/2015/491567491567Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care SettingNeda Hashemi-Sadraei0Pimprapa Vejpongsa1Muhamed Baljevic2Lei Chen3Modupe Idowu4Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX 77030, USADepartment of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX 77030, USADivision of Cancer Medicine, The University of Texas, MD Anderson Cancer Center, Houston, TX 77030, USADepartment of Pathology and Laboratory Medicine, University of Texas Health Science Center at Houston, Houston, TX 77030, USADepartment of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX 77030, USAHemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. As clinical and laboratory features of HLH could overlap with septic shock syndrome in most patients, the diagnosis of HLH, especially in adults, is the most challenging aspect of the disease that results in delayed recognition and treatment of rapidly progressive multiorgan system failure. We report a case of Hemophagocytic lymphohistiocytosis in a patient who presented with signs of septic shock syndrome and we review the literature on the topic.http://dx.doi.org/10.1155/2015/491567
spellingShingle Neda Hashemi-Sadraei
Pimprapa Vejpongsa
Muhamed Baljevic
Lei Chen
Modupe Idowu
Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting
Case Reports in Hematology
title Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting
title_full Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting
title_fullStr Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting
title_full_unstemmed Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting
title_short Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting
title_sort epstein barr virus related hemophagocytic lymphohistiocytosis hematologic emergency in the critical care setting
url http://dx.doi.org/10.1155/2015/491567
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