Hemophagocytic lymphohistiocytosis: an update for nephrologists
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response sy...
Ausführliche Beschreibung
Autor*in: |
Filippone, Edward J. [verfasserIn] |
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E-Artikel |
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Sprache: |
Englisch |
Erschienen: |
2016 |
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Schlagwörter: |
Hemophagocytic lymphohistiocytosis |
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Anmerkung: |
© Springer Science+Business Media Dordrecht 2016 |
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Übergeordnetes Werk: |
Enthalten in: International urology and nephrology - Dordrecht [u.a.] : Springer Science + Business Media B.V., 1969, 48(2016), 8 vom: 20. Apr., Seite 1291-1304 |
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Übergeordnetes Werk: |
volume:48 ; year:2016 ; number:8 ; day:20 ; month:04 ; pages:1291-1304 |
Links: |
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DOI / URN: |
10.1007/s11255-016-1294-z |
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Katalog-ID: |
SPR018264492 |
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520 | |a Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. | ||
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650 | 4 | |a Systemic inflammatory response syndrome |7 (dpeaa)DE-He213 | |
700 | 1 | |a Farber, John L. |4 aut | |
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10.1007/s11255-016-1294-z doi (DE-627)SPR018264492 (SPR)s11255-016-1294-z-e DE-627 ger DE-627 rakwb eng Filippone, Edward J. verfasserin aut Hemophagocytic lymphohistiocytosis: an update for nephrologists 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Science+Business Media Dordrecht 2016 Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic syndrome (dpeaa)DE-He213 Acute kidney injury (dpeaa)DE-He213 Kidney transplantation (dpeaa)DE-He213 Systemic inflammatory response syndrome (dpeaa)DE-He213 Farber, John L. aut Enthalten in International urology and nephrology Dordrecht [u.a.] : Springer Science + Business Media B.V., 1969 48(2016), 8 vom: 20. Apr., Seite 1291-1304 (DE-627)320529134 (DE-600)2015547-5 1573-2584 nnns volume:48 year:2016 number:8 day:20 month:04 pages:1291-1304 https://dx.doi.org/10.1007/s11255-016-1294-z lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 48 2016 8 20 04 1291-1304 |
spelling |
10.1007/s11255-016-1294-z doi (DE-627)SPR018264492 (SPR)s11255-016-1294-z-e DE-627 ger DE-627 rakwb eng Filippone, Edward J. verfasserin aut Hemophagocytic lymphohistiocytosis: an update for nephrologists 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Science+Business Media Dordrecht 2016 Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic syndrome (dpeaa)DE-He213 Acute kidney injury (dpeaa)DE-He213 Kidney transplantation (dpeaa)DE-He213 Systemic inflammatory response syndrome (dpeaa)DE-He213 Farber, John L. aut Enthalten in International urology and nephrology Dordrecht [u.a.] : Springer Science + Business Media B.V., 1969 48(2016), 8 vom: 20. Apr., Seite 1291-1304 (DE-627)320529134 (DE-600)2015547-5 1573-2584 nnns volume:48 year:2016 number:8 day:20 month:04 pages:1291-1304 https://dx.doi.org/10.1007/s11255-016-1294-z lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 48 2016 8 20 04 1291-1304 |
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10.1007/s11255-016-1294-z doi (DE-627)SPR018264492 (SPR)s11255-016-1294-z-e DE-627 ger DE-627 rakwb eng Filippone, Edward J. verfasserin aut Hemophagocytic lymphohistiocytosis: an update for nephrologists 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Science+Business Media Dordrecht 2016 Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic syndrome (dpeaa)DE-He213 Acute kidney injury (dpeaa)DE-He213 Kidney transplantation (dpeaa)DE-He213 Systemic inflammatory response syndrome (dpeaa)DE-He213 Farber, John L. aut Enthalten in International urology and nephrology Dordrecht [u.a.] : Springer Science + Business Media B.V., 1969 48(2016), 8 vom: 20. Apr., Seite 1291-1304 (DE-627)320529134 (DE-600)2015547-5 1573-2584 nnns volume:48 year:2016 number:8 day:20 month:04 pages:1291-1304 https://dx.doi.org/10.1007/s11255-016-1294-z lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 48 2016 8 20 04 1291-1304 |
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10.1007/s11255-016-1294-z doi (DE-627)SPR018264492 (SPR)s11255-016-1294-z-e DE-627 ger DE-627 rakwb eng Filippone, Edward J. verfasserin aut Hemophagocytic lymphohistiocytosis: an update for nephrologists 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Science+Business Media Dordrecht 2016 Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic syndrome (dpeaa)DE-He213 Acute kidney injury (dpeaa)DE-He213 Kidney transplantation (dpeaa)DE-He213 Systemic inflammatory response syndrome (dpeaa)DE-He213 Farber, John L. aut Enthalten in International urology and nephrology Dordrecht [u.a.] : Springer Science + Business Media B.V., 1969 48(2016), 8 vom: 20. Apr., Seite 1291-1304 (DE-627)320529134 (DE-600)2015547-5 1573-2584 nnns volume:48 year:2016 number:8 day:20 month:04 pages:1291-1304 https://dx.doi.org/10.1007/s11255-016-1294-z lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 48 2016 8 20 04 1291-1304 |
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10.1007/s11255-016-1294-z doi (DE-627)SPR018264492 (SPR)s11255-016-1294-z-e DE-627 ger DE-627 rakwb eng Filippone, Edward J. verfasserin aut Hemophagocytic lymphohistiocytosis: an update for nephrologists 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Science+Business Media Dordrecht 2016 Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic syndrome (dpeaa)DE-He213 Acute kidney injury (dpeaa)DE-He213 Kidney transplantation (dpeaa)DE-He213 Systemic inflammatory response syndrome (dpeaa)DE-He213 Farber, John L. aut Enthalten in International urology and nephrology Dordrecht [u.a.] : Springer Science + Business Media B.V., 1969 48(2016), 8 vom: 20. Apr., Seite 1291-1304 (DE-627)320529134 (DE-600)2015547-5 1573-2584 nnns volume:48 year:2016 number:8 day:20 month:04 pages:1291-1304 https://dx.doi.org/10.1007/s11255-016-1294-z lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 48 2016 8 20 04 1291-1304 |
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Filippone, Edward J. |
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Filippone, Edward J. misc Hemophagocytic lymphohistiocytosis misc Macrophage activation syndrome misc Hemophagocytic syndrome misc Acute kidney injury misc Kidney transplantation misc Systemic inflammatory response syndrome Hemophagocytic lymphohistiocytosis: an update for nephrologists |
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Hemophagocytic lymphohistiocytosis: an update for nephrologists Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic syndrome (dpeaa)DE-He213 Acute kidney injury (dpeaa)DE-He213 Kidney transplantation (dpeaa)DE-He213 Systemic inflammatory response syndrome (dpeaa)DE-He213 |
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hemophagocytic lymphohistiocytosis: an update for nephrologists |
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Hemophagocytic lymphohistiocytosis: an update for nephrologists |
abstract |
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. © Springer Science+Business Media Dordrecht 2016 |
abstractGer |
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. © Springer Science+Business Media Dordrecht 2016 |
abstract_unstemmed |
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed. © Springer Science+Business Media Dordrecht 2016 |
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title_short |
Hemophagocytic lymphohistiocytosis: an update for nephrologists |
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https://dx.doi.org/10.1007/s11255-016-1294-z |
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Farber, John L. |
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2024-07-03T18:33:02.085Z |
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|
score |
7.3986425 |