Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening condition characterized by immune dysregulation and benign proliferation of phagocytic cells, macrophages, and histiocytes. Lymphocytes (CD8 T-cells and natural killer cells) also play a major role in HLH. The ensuing cytokine stor...
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Gespeichert in:

Autor*in:	Rabab Taha [verfasserIn] Fahmi Al-Dhaheri [verfasserIn] Naji Dwid [verfasserIn] Murad R Almowarey [verfasserIn] Maged Al-Ammari [verfasserIn] Haytham Abbas [verfasserIn] Abdulrahman Alamoudi [verfasserIn] Omar Fathaldin [verfasserIn] Tarek Mohamed [verfasserIn] Hani Almoallim [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2017

Schlagwörter:	Hemophagocytic lymphohistiocytosis syndrome hemophagocytic syndrome lymphohistiocytosis

Übergeordnetes Werk:	In: Journal of Applied Hematology - Wolters Kluwer Medknow Publications, 2018, 8(2017), 4, Seite 145-151
Übergeordnetes Werk:	volume:8 ; year:2017 ; number:4 ; pages:145-151

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.4103/joah.joah_42_17

Katalog-ID:	DOAJ03461415X

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author	Rabab Taha
spellingShingle	Rabab Taha misc RC666-701 misc Hemophagocytic lymphohistiocytosis syndrome misc hemophagocytic syndrome misc lymphohistiocytosis misc Diseases of the circulatory (Cardiovascular) system Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature
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topic_title	RC666-701 Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature Hemophagocytic lymphohistiocytosis syndrome hemophagocytic syndrome lymphohistiocytosis
topic	misc RC666-701 misc Hemophagocytic lymphohistiocytosis syndrome misc hemophagocytic syndrome misc lymphohistiocytosis misc Diseases of the circulatory (Cardiovascular) system
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title	Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature
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title_full	Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature
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author_browse	Rabab Taha Fahmi Al-Dhaheri Naji Dwid Murad R Almowarey Maged Al-Ammari Haytham Abbas Abdulrahman Alamoudi Omar Fathaldin Tarek Mohamed Hani Almoallim
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title_sort	secondary hemophagocytic lymphohistiocytosis syndrome in adults: a case series and review of the literature
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title_auth	Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature
abstract	Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening condition characterized by immune dysregulation and benign proliferation of phagocytic cells, macrophages, and histiocytes. Lymphocytes (CD8 T-cells and natural killer cells) also play a major role in HLH. The ensuing cytokine storm and blood cells phagocytosis can lead to fatal multiple organ failure. So any diseases that can lead to activation of these cytokines can lead to hemophagocytic syndrome. 5 adult patients presented to Dr. Soliman Fakeeh Hospital, Jeddah, Saudi Arabia during 2014–2016 and fulfill the diagnostic criteria of HLH according to HLH-2004 diagnostic and therapeutic guidelines for HLH , have been included in this case series. Two out of five had viral infection, one patient was diagnosed with tuberculosis, and one patient developed secondary HLH in due to malignancy. The fifth patient did not have an identiable etiology. All of our patients presented with different symptoms and were diagnosed based on the standard approved criteria. Only two Out of five patients survived and remained disease free for a follow-up period of 24 months. As a conclusion, HLH syndrome is a rare condition with a high mortality rate. Aggressive treatment approach and early Intensive Care Unit admission is strongly recommended in patients with the cardinal diagnostic features of this condition.
abstractGer	Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening condition characterized by immune dysregulation and benign proliferation of phagocytic cells, macrophages, and histiocytes. Lymphocytes (CD8 T-cells and natural killer cells) also play a major role in HLH. The ensuing cytokine storm and blood cells phagocytosis can lead to fatal multiple organ failure. So any diseases that can lead to activation of these cytokines can lead to hemophagocytic syndrome. 5 adult patients presented to Dr. Soliman Fakeeh Hospital, Jeddah, Saudi Arabia during 2014–2016 and fulfill the diagnostic criteria of HLH according to HLH-2004 diagnostic and therapeutic guidelines for HLH , have been included in this case series. Two out of five had viral infection, one patient was diagnosed with tuberculosis, and one patient developed secondary HLH in due to malignancy. The fifth patient did not have an identiable etiology. All of our patients presented with different symptoms and were diagnosed based on the standard approved criteria. Only two Out of five patients survived and remained disease free for a follow-up period of 24 months. As a conclusion, HLH syndrome is a rare condition with a high mortality rate. Aggressive treatment approach and early Intensive Care Unit admission is strongly recommended in patients with the cardinal diagnostic features of this condition.
abstract_unstemmed	Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening condition characterized by immune dysregulation and benign proliferation of phagocytic cells, macrophages, and histiocytes. Lymphocytes (CD8 T-cells and natural killer cells) also play a major role in HLH. The ensuing cytokine storm and blood cells phagocytosis can lead to fatal multiple organ failure. So any diseases that can lead to activation of these cytokines can lead to hemophagocytic syndrome. 5 adult patients presented to Dr. Soliman Fakeeh Hospital, Jeddah, Saudi Arabia during 2014–2016 and fulfill the diagnostic criteria of HLH according to HLH-2004 diagnostic and therapeutic guidelines for HLH , have been included in this case series. Two out of five had viral infection, one patient was diagnosed with tuberculosis, and one patient developed secondary HLH in due to malignancy. The fifth patient did not have an identiable etiology. All of our patients presented with different symptoms and were diagnosed based on the standard approved criteria. Only two Out of five patients survived and remained disease free for a follow-up period of 24 months. As a conclusion, HLH syndrome is a rare condition with a high mortality rate. Aggressive treatment approach and early Intensive Care Unit admission is strongly recommended in patients with the cardinal diagnostic features of this condition.
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title_short	Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature
url	https://doi.org/10.4103/joah.joah_42_17 https://doaj.org/article/9cc4c67906504c1eafe5951fb157d071 http://www.jahjournal.org/article.asp?issn=1658-5127;year=2017;volume=8;issue=4;spage=145;epage=151;aulast=Taha https://doaj.org/toc/1658-5127
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author2	Fahmi Al-Dhaheri Naji Dwid Murad R Almowarey Maged Al-Ammari Haytham Abbas Abdulrahman Alamoudi Omar Fathaldin Tarek Mohamed Hani Almoallim
author2Str	Fahmi Al-Dhaheri Naji Dwid Murad R Almowarey Maged Al-Ammari Haytham Abbas Abdulrahman Alamoudi Omar Fathaldin Tarek Mohamed Hani Almoallim
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up_date	2024-07-03T23:56:33.598Z
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code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Hani Almoallim</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">In</subfield><subfield code="t">Journal of Applied Hematology</subfield><subfield code="d">Wolters Kluwer Medknow Publications, 2018</subfield><subfield code="g">8(2017), 4, Seite 145-151</subfield><subfield code="w">(DE-627)768576431</subfield><subfield code="w">(DE-600)2733725-X</subfield><subfield code="x">24546976</subfield><subfield code="7">nnns</subfield></datafield><datafield tag="773" ind1="1" ind2="8"><subfield code="g">volume:8</subfield><subfield code="g">year:2017</subfield><subfield code="g">number:4</subfield><subfield code="g">pages:145-151</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://doi.org/10.4103/joah.joah_42_17</subfield><subfield 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Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature

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