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...
Ausführliche Beschreibung
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] |
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Format: |
E-Artikel |
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Sprache: |
Englisch |
Erschienen: |
2017 |
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Schlagwörter: |
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Übergeordnetes Werk: |
In: Journal of Applied Hematology - Wolters Kluwer Medknow Publications, 2018, 8(2017), 4, Seite 145-151 |
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Übergeordnetes Werk: |
volume:8 ; year:2017 ; number:4 ; pages:145-151 |
Links: |
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DOI / URN: |
10.4103/joah.joah_42_17 |
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Katalog-ID: |
DOAJ03461415X |
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10.4103/joah.joah_42_17 doi (DE-627)DOAJ03461415X (DE-599)DOAJ9cc4c67906504c1eafe5951fb157d071 DE-627 ger DE-627 rakwb eng RC666-701 Rabab Taha verfasserin aut Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. Hemophagocytic lymphohistiocytosis syndrome hemophagocytic syndrome lymphohistiocytosis Diseases of the circulatory (Cardiovascular) system Fahmi Al-Dhaheri verfasserin aut Naji Dwid verfasserin aut Murad R Almowarey verfasserin aut Maged Al-Ammari verfasserin aut Haytham Abbas verfasserin aut Abdulrahman Alamoudi verfasserin aut Omar Fathaldin verfasserin aut Tarek Mohamed verfasserin aut Hani Almoallim verfasserin aut In Journal of Applied Hematology Wolters Kluwer Medknow Publications, 2018 8(2017), 4, Seite 145-151 (DE-627)768576431 (DE-600)2733725-X 24546976 nnns volume:8 year:2017 number:4 pages:145-151 https://doi.org/10.4103/joah.joah_42_17 kostenfrei https://doaj.org/article/9cc4c67906504c1eafe5951fb157d071 kostenfrei http://www.jahjournal.org/article.asp?issn=1658-5127;year=2017;volume=8;issue=4;spage=145;epage=151;aulast=Taha kostenfrei https://doaj.org/toc/1658-5127 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2017 4 145-151 |
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10.4103/joah.joah_42_17 doi (DE-627)DOAJ03461415X (DE-599)DOAJ9cc4c67906504c1eafe5951fb157d071 DE-627 ger DE-627 rakwb eng RC666-701 Rabab Taha verfasserin aut Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. Hemophagocytic lymphohistiocytosis syndrome hemophagocytic syndrome lymphohistiocytosis Diseases of the circulatory (Cardiovascular) system Fahmi Al-Dhaheri verfasserin aut Naji Dwid verfasserin aut Murad R Almowarey verfasserin aut Maged Al-Ammari verfasserin aut Haytham Abbas verfasserin aut Abdulrahman Alamoudi verfasserin aut Omar Fathaldin verfasserin aut Tarek Mohamed verfasserin aut Hani Almoallim verfasserin aut In Journal of Applied Hematology Wolters Kluwer Medknow Publications, 2018 8(2017), 4, Seite 145-151 (DE-627)768576431 (DE-600)2733725-X 24546976 nnns volume:8 year:2017 number:4 pages:145-151 https://doi.org/10.4103/joah.joah_42_17 kostenfrei https://doaj.org/article/9cc4c67906504c1eafe5951fb157d071 kostenfrei http://www.jahjournal.org/article.asp?issn=1658-5127;year=2017;volume=8;issue=4;spage=145;epage=151;aulast=Taha kostenfrei https://doaj.org/toc/1658-5127 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2017 4 145-151 |
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10.4103/joah.joah_42_17 doi (DE-627)DOAJ03461415X (DE-599)DOAJ9cc4c67906504c1eafe5951fb157d071 DE-627 ger DE-627 rakwb eng RC666-701 Rabab Taha verfasserin aut Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. Hemophagocytic lymphohistiocytosis syndrome hemophagocytic syndrome lymphohistiocytosis Diseases of the circulatory (Cardiovascular) system Fahmi Al-Dhaheri verfasserin aut Naji Dwid verfasserin aut Murad R Almowarey verfasserin aut Maged Al-Ammari verfasserin aut Haytham Abbas verfasserin aut Abdulrahman Alamoudi verfasserin aut Omar Fathaldin verfasserin aut Tarek Mohamed verfasserin aut Hani Almoallim verfasserin aut In Journal of Applied Hematology Wolters Kluwer Medknow Publications, 2018 8(2017), 4, Seite 145-151 (DE-627)768576431 (DE-600)2733725-X 24546976 nnns volume:8 year:2017 number:4 pages:145-151 https://doi.org/10.4103/joah.joah_42_17 kostenfrei https://doaj.org/article/9cc4c67906504c1eafe5951fb157d071 kostenfrei http://www.jahjournal.org/article.asp?issn=1658-5127;year=2017;volume=8;issue=4;spage=145;epage=151;aulast=Taha kostenfrei https://doaj.org/toc/1658-5127 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2017 4 145-151 |
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10.4103/joah.joah_42_17 doi (DE-627)DOAJ03461415X (DE-599)DOAJ9cc4c67906504c1eafe5951fb157d071 DE-627 ger DE-627 rakwb eng RC666-701 Rabab Taha verfasserin aut Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. Hemophagocytic lymphohistiocytosis syndrome hemophagocytic syndrome lymphohistiocytosis Diseases of the circulatory (Cardiovascular) system Fahmi Al-Dhaheri verfasserin aut Naji Dwid verfasserin aut Murad R Almowarey verfasserin aut Maged Al-Ammari verfasserin aut Haytham Abbas verfasserin aut Abdulrahman Alamoudi verfasserin aut Omar Fathaldin verfasserin aut Tarek Mohamed verfasserin aut Hani Almoallim verfasserin aut In Journal of Applied Hematology Wolters Kluwer Medknow Publications, 2018 8(2017), 4, Seite 145-151 (DE-627)768576431 (DE-600)2733725-X 24546976 nnns volume:8 year:2017 number:4 pages:145-151 https://doi.org/10.4103/joah.joah_42_17 kostenfrei https://doaj.org/article/9cc4c67906504c1eafe5951fb157d071 kostenfrei http://www.jahjournal.org/article.asp?issn=1658-5127;year=2017;volume=8;issue=4;spage=145;epage=151;aulast=Taha kostenfrei https://doaj.org/toc/1658-5127 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 8 2017 4 145-151 |
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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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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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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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ind1="0" ind2=" "><subfield code="a">Murad R Almowarey</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Maged Al-Ammari</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Haytham Abbas</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Abdulrahman Alamoudi</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Omar Fathaldin</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Tarek Mohamed</subfield><subfield code="e">verfasserin</subfield><subfield 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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