A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients
Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two case...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Hori, Masayuki [verfasserIn] Yasumi, Takahiro [verfasserIn] Shimodera, Saeko [verfasserIn] Shibata, Hirofumi [verfasserIn] Hiejima, Eitaro [verfasserIn] Oda, Hirotsugu [verfasserIn] Izawa, Kazushi [verfasserIn] Kawai, Tomoki [verfasserIn] Ishimura, Masataka [verfasserIn] Nakano, Naoko [verfasserIn] Shirakawa, Ryutaro [verfasserIn] Nishikomori, Ryuta [verfasserIn] Takada, Hidetoshi [verfasserIn] Morita, Satoshi [verfasserIn] Horiuchi, Hisanori [verfasserIn] Ohara, Osamu [verfasserIn] Ishii, Eiichi [verfasserIn] Heike, Toshio [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2016 |
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| Schlagwörter: |
Familial hemophagocytic lymphohistiocytosis type 3 |
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| Übergeordnetes Werk: |
Enthalten in: Journal of clinical immunology - Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981, 37(2016), 1 vom: 28. Nov., Seite 92-99 |
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| Übergeordnetes Werk: |
volume:37 ; year:2016 ; number:1 ; day:28 ; month:11 ; pages:92-99 |
| Links: |
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| DOI / URN: |
10.1007/s10875-016-0357-3 |
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| Katalog-ID: |
SPR014246627 |
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| 100 | 1 | |a Hori, Masayuki |e verfasserin |4 aut | |
| 245 | 1 | 2 | |a A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients |
| 264 | 1 | |c 2016 | |
| 336 | |a Text |b txt |2 rdacontent | ||
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| 520 | |a Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. | ||
| 650 | 4 | |a Familial hemophagocytic lymphohistiocytosis type 3 |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a lysosomal degranulation defect |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a functional screening assay |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Yasumi, Takahiro |e verfasserin |4 aut | |
| 700 | 1 | |a Shimodera, Saeko |e verfasserin |4 aut | |
| 700 | 1 | |a Shibata, Hirofumi |e verfasserin |4 aut | |
| 700 | 1 | |a Hiejima, Eitaro |e verfasserin |4 aut | |
| 700 | 1 | |a Oda, Hirotsugu |e verfasserin |4 aut | |
| 700 | 1 | |a Izawa, Kazushi |e verfasserin |4 aut | |
| 700 | 1 | |a Kawai, Tomoki |e verfasserin |4 aut | |
| 700 | 1 | |a Ishimura, Masataka |e verfasserin |4 aut | |
| 700 | 1 | |a Nakano, Naoko |e verfasserin |4 aut | |
| 700 | 1 | |a Shirakawa, Ryutaro |e verfasserin |4 aut | |
| 700 | 1 | |a Nishikomori, Ryuta |e verfasserin |4 aut | |
| 700 | 1 | |a Takada, Hidetoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Morita, Satoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Horiuchi, Hisanori |e verfasserin |4 aut | |
| 700 | 1 | |a Ohara, Osamu |e verfasserin |4 aut | |
| 700 | 1 | |a Ishii, Eiichi |e verfasserin |4 aut | |
| 700 | 1 | |a Heike, Toshio |e verfasserin |4 aut | |
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10.1007/s10875-016-0357-3 doi (DE-627)SPR014246627 (SPR)s10875-016-0357-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Hori, Masayuki verfasserin aut A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. Familial hemophagocytic lymphohistiocytosis type 3 (dpeaa)DE-He213 lysosomal degranulation defect (dpeaa)DE-He213 functional screening assay (dpeaa)DE-He213 Yasumi, Takahiro verfasserin aut Shimodera, Saeko verfasserin aut Shibata, Hirofumi verfasserin aut Hiejima, Eitaro verfasserin aut Oda, Hirotsugu verfasserin aut Izawa, Kazushi verfasserin aut Kawai, Tomoki verfasserin aut Ishimura, Masataka verfasserin aut Nakano, Naoko verfasserin aut Shirakawa, Ryutaro verfasserin aut Nishikomori, Ryuta verfasserin aut Takada, Hidetoshi verfasserin aut Morita, Satoshi verfasserin aut Horiuchi, Hisanori verfasserin aut Ohara, Osamu verfasserin aut Ishii, Eiichi verfasserin aut Heike, Toshio verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 37(2016), 1 vom: 28. Nov., Seite 92-99 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:37 year:2016 number:1 day:28 month:11 pages:92-99 https://dx.doi.org/10.1007/s10875-016-0357-3 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_152 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_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_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.45 ASE AR 37 2016 1 28 11 92-99 |
| spelling |
10.1007/s10875-016-0357-3 doi (DE-627)SPR014246627 (SPR)s10875-016-0357-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Hori, Masayuki verfasserin aut A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. Familial hemophagocytic lymphohistiocytosis type 3 (dpeaa)DE-He213 lysosomal degranulation defect (dpeaa)DE-He213 functional screening assay (dpeaa)DE-He213 Yasumi, Takahiro verfasserin aut Shimodera, Saeko verfasserin aut Shibata, Hirofumi verfasserin aut Hiejima, Eitaro verfasserin aut Oda, Hirotsugu verfasserin aut Izawa, Kazushi verfasserin aut Kawai, Tomoki verfasserin aut Ishimura, Masataka verfasserin aut Nakano, Naoko verfasserin aut Shirakawa, Ryutaro verfasserin aut Nishikomori, Ryuta verfasserin aut Takada, Hidetoshi verfasserin aut Morita, Satoshi verfasserin aut Horiuchi, Hisanori verfasserin aut Ohara, Osamu verfasserin aut Ishii, Eiichi verfasserin aut Heike, Toshio verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 37(2016), 1 vom: 28. Nov., Seite 92-99 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:37 year:2016 number:1 day:28 month:11 pages:92-99 https://dx.doi.org/10.1007/s10875-016-0357-3 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_152 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_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_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.45 ASE AR 37 2016 1 28 11 92-99 |
| allfields_unstemmed |
10.1007/s10875-016-0357-3 doi (DE-627)SPR014246627 (SPR)s10875-016-0357-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Hori, Masayuki verfasserin aut A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. Familial hemophagocytic lymphohistiocytosis type 3 (dpeaa)DE-He213 lysosomal degranulation defect (dpeaa)DE-He213 functional screening assay (dpeaa)DE-He213 Yasumi, Takahiro verfasserin aut Shimodera, Saeko verfasserin aut Shibata, Hirofumi verfasserin aut Hiejima, Eitaro verfasserin aut Oda, Hirotsugu verfasserin aut Izawa, Kazushi verfasserin aut Kawai, Tomoki verfasserin aut Ishimura, Masataka verfasserin aut Nakano, Naoko verfasserin aut Shirakawa, Ryutaro verfasserin aut Nishikomori, Ryuta verfasserin aut Takada, Hidetoshi verfasserin aut Morita, Satoshi verfasserin aut Horiuchi, Hisanori verfasserin aut Ohara, Osamu verfasserin aut Ishii, Eiichi verfasserin aut Heike, Toshio verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 37(2016), 1 vom: 28. Nov., Seite 92-99 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:37 year:2016 number:1 day:28 month:11 pages:92-99 https://dx.doi.org/10.1007/s10875-016-0357-3 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_152 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_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_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.45 ASE AR 37 2016 1 28 11 92-99 |
| allfieldsGer |
10.1007/s10875-016-0357-3 doi (DE-627)SPR014246627 (SPR)s10875-016-0357-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Hori, Masayuki verfasserin aut A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. Familial hemophagocytic lymphohistiocytosis type 3 (dpeaa)DE-He213 lysosomal degranulation defect (dpeaa)DE-He213 functional screening assay (dpeaa)DE-He213 Yasumi, Takahiro verfasserin aut Shimodera, Saeko verfasserin aut Shibata, Hirofumi verfasserin aut Hiejima, Eitaro verfasserin aut Oda, Hirotsugu verfasserin aut Izawa, Kazushi verfasserin aut Kawai, Tomoki verfasserin aut Ishimura, Masataka verfasserin aut Nakano, Naoko verfasserin aut Shirakawa, Ryutaro verfasserin aut Nishikomori, Ryuta verfasserin aut Takada, Hidetoshi verfasserin aut Morita, Satoshi verfasserin aut Horiuchi, Hisanori verfasserin aut Ohara, Osamu verfasserin aut Ishii, Eiichi verfasserin aut Heike, Toshio verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 37(2016), 1 vom: 28. Nov., Seite 92-99 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:37 year:2016 number:1 day:28 month:11 pages:92-99 https://dx.doi.org/10.1007/s10875-016-0357-3 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_152 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_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_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.45 ASE AR 37 2016 1 28 11 92-99 |
| allfieldsSound |
10.1007/s10875-016-0357-3 doi (DE-627)SPR014246627 (SPR)s10875-016-0357-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Hori, Masayuki verfasserin aut A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. Familial hemophagocytic lymphohistiocytosis type 3 (dpeaa)DE-He213 lysosomal degranulation defect (dpeaa)DE-He213 functional screening assay (dpeaa)DE-He213 Yasumi, Takahiro verfasserin aut Shimodera, Saeko verfasserin aut Shibata, Hirofumi verfasserin aut Hiejima, Eitaro verfasserin aut Oda, Hirotsugu verfasserin aut Izawa, Kazushi verfasserin aut Kawai, Tomoki verfasserin aut Ishimura, Masataka verfasserin aut Nakano, Naoko verfasserin aut Shirakawa, Ryutaro verfasserin aut Nishikomori, Ryuta verfasserin aut Takada, Hidetoshi verfasserin aut Morita, Satoshi verfasserin aut Horiuchi, Hisanori verfasserin aut Ohara, Osamu verfasserin aut Ishii, Eiichi verfasserin aut Heike, Toshio verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 37(2016), 1 vom: 28. Nov., Seite 92-99 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:37 year:2016 number:1 day:28 month:11 pages:92-99 https://dx.doi.org/10.1007/s10875-016-0357-3 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_152 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_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_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.45 ASE AR 37 2016 1 28 11 92-99 |
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Enthalten in Journal of clinical immunology 37(2016), 1 vom: 28. Nov., Seite 92-99 volume:37 year:2016 number:1 day:28 month:11 pages:92-99 |
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Enthalten in Journal of clinical immunology 37(2016), 1 vom: 28. Nov., Seite 92-99 volume:37 year:2016 number:1 day:28 month:11 pages:92-99 |
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Familial hemophagocytic lymphohistiocytosis type 3 lysosomal degranulation defect functional screening assay |
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Hori, Masayuki @@aut@@ Yasumi, Takahiro @@aut@@ Shimodera, Saeko @@aut@@ Shibata, Hirofumi @@aut@@ Hiejima, Eitaro @@aut@@ Oda, Hirotsugu @@aut@@ Izawa, Kazushi @@aut@@ Kawai, Tomoki @@aut@@ Ishimura, Masataka @@aut@@ Nakano, Naoko @@aut@@ Shirakawa, Ryutaro @@aut@@ Nishikomori, Ryuta @@aut@@ Takada, Hidetoshi @@aut@@ Morita, Satoshi @@aut@@ Horiuchi, Hisanori @@aut@@ Ohara, Osamu @@aut@@ Ishii, Eiichi @@aut@@ Heike, Toshio @@aut@@ |
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2016-11-28T00:00:00Z |
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320573362 |
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3610 |
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SPR014246627 |
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englisch |
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It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. 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| author |
Hori, Masayuki |
| spellingShingle |
Hori, Masayuki ddc 610 bkl 44.45 misc Familial hemophagocytic lymphohistiocytosis type 3 misc lysosomal degranulation defect misc functional screening assay A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients |
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1573-2592 |
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610 ASE 44.45 bkl A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients Familial hemophagocytic lymphohistiocytosis type 3 (dpeaa)DE-He213 lysosomal degranulation defect (dpeaa)DE-He213 functional screening assay (dpeaa)DE-He213 |
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ddc 610 bkl 44.45 misc Familial hemophagocytic lymphohistiocytosis type 3 misc lysosomal degranulation defect misc functional screening assay |
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ddc 610 bkl 44.45 misc Familial hemophagocytic lymphohistiocytosis type 3 misc lysosomal degranulation defect misc functional screening assay |
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ddc 610 bkl 44.45 misc Familial hemophagocytic lymphohistiocytosis type 3 misc lysosomal degranulation defect misc functional screening assay |
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Journal of clinical immunology |
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Journal of clinical immunology |
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A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients |
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(DE-627)SPR014246627 (SPR)s10875-016-0357-3-e |
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A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients |
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Hori, Masayuki |
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Journal of clinical immunology |
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Journal of clinical immunology |
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2016 |
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Hori, Masayuki Yasumi, Takahiro Shimodera, Saeko Shibata, Hirofumi Hiejima, Eitaro Oda, Hirotsugu Izawa, Kazushi Kawai, Tomoki Ishimura, Masataka Nakano, Naoko Shirakawa, Ryutaro Nishikomori, Ryuta Takada, Hidetoshi Morita, Satoshi Horiuchi, Hisanori Ohara, Osamu Ishii, Eiichi Heike, Toshio |
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Hori, Masayuki |
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10.1007/s10875-016-0357-3 |
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610 |
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verfasserin |
| title_sort |
$ cd57^{+} $ ctl degranulation assay effectively identifies familial hemophagocytic lymphohistiocytosis type 3 patients |
| title_auth |
A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients |
| abstract |
Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. |
| abstractGer |
Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. |
| abstract_unstemmed |
Purpose Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in these cases, the degranulation assays using freshly isolated and interleukin (IL)-2-activated NK cells yielded contradictory results. Since the defective degranulation of $ CD57^{+} $ cytotoxic T lymphocytes (CTLs) in these cases was helpful for making the diagnosis, we assessed whether the $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell assays. Methods Forty additional patients with hemophagocytic lymphohistiocytosis were prospectively screened for FHL3 by measuring the perforin expression in NK cells and the expression of Munc13-4, syntaxin-11, and Munc18-2 in platelets and by performing NK cell and CTL degranulation assays. The results were confirmed by genetic analysis. Results The freshly isolated NK cell degranulation assay detected FHL3 patients with high sensitivity (100%) but low specificity (71%). The IL-2-stimulated NK cell assay had improved specificity, but 3 out of the 31 non-FHL3 patients still showed degranulation below the threshold level. The $ CD57^{+} $ CTL degranulation assay identified FHL3 patients with high sensitivity and specificity (both 100%). Conclusions The $ CD57^{+} $ CTL degranulation assay more effectively identified FHL3 patients than the NK cell-based assays. |
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| title_short |
A $ CD57^{+} $ CTL Degranulation Assay Effectively Identifies Familial Hemophagocytic Lymphohistiocytosis Type 3 Patients |
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https://dx.doi.org/10.1007/s10875-016-0357-3 |
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Yasumi, Takahiro Shimodera, Saeko Shibata, Hirofumi Hiejima, Eitaro Oda, Hirotsugu Izawa, Kazushi Kawai, Tomoki Ishimura, Masataka Nakano, Naoko Shirakawa, Ryutaro Nishikomori, Ryuta Takada, Hidetoshi Morita, Satoshi Horiuchi, Hisanori Ohara, Osamu Ishii, Eiichi Heike, Toshio |
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Yasumi, Takahiro Shimodera, Saeko Shibata, Hirofumi Hiejima, Eitaro Oda, Hirotsugu Izawa, Kazushi Kawai, Tomoki Ishimura, Masataka Nakano, Naoko Shirakawa, Ryutaro Nishikomori, Ryuta Takada, Hidetoshi Morita, Satoshi Horiuchi, Hisanori Ohara, Osamu Ishii, Eiichi Heike, Toshio |
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10.1007/s10875-016-0357-3 |
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2024-07-04T00:50:23.939Z |
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| score |
7.4005184 |