Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis
Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Wolska-Kuśnierz, Beata [verfasserIn] Gregorek, Hanna [verfasserIn] Chrzanowska, Krystyna [verfasserIn] Piątosa, Barbara [verfasserIn] Pietrucha, Barbara [verfasserIn] Heropolitańska-Pliszka, Edyta [verfasserIn] Pac, Małgorzata [verfasserIn] Klaudel-Dreszler, Maja [verfasserIn] Kostyuchenko, Larysa [verfasserIn] Pasic, Srdjan [verfasserIn] Marodi, Laszlo [verfasserIn] Belohradsky, Bernd H. [verfasserIn] Čižnár, Peter [verfasserIn] Shcherbina, Anna [verfasserIn] Kilic, Sara Sebnem [verfasserIn] Baumann, Ulrich [verfasserIn] Seidel, Markus G. [verfasserIn] Gennery, Andrew R. [verfasserIn] Syczewska, Małgorzata [verfasserIn] Mikołuć, Bożena [verfasserIn] Kałwak, Krzysztof [verfasserIn] Styczyński, Jan [verfasserIn] Pieczonka, Anna [verfasserIn] Drabko, Katarzyna [verfasserIn] Wakulińska, Anna [verfasserIn] Gathmann, Benjamin [verfasserIn] Albert, Michael H. [verfasserIn] Skarżyńska, Urszula [verfasserIn] Bernatowska, Ewa [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2015 |
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| Schlagwörter: |
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| Übergeordnetes Werk: |
Enthalten in: Journal of clinical immunology - Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981, 35(2015), 6 vom: 01. Aug., Seite 538-549 |
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| Übergeordnetes Werk: |
volume:35 ; year:2015 ; number:6 ; day:01 ; month:08 ; pages:538-549 |
| Links: |
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| DOI / URN: |
10.1007/s10875-015-0186-9 |
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SPR014244950 |
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| 245 | 1 | 0 | |a Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis |
| 264 | 1 | |c 2015 | |
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| 520 | |a Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. | ||
| 650 | 4 | |a Nijmegen breakage syndrome |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a primary immunodeficiencies |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a chromosomal instability |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a hematopoietic stem cell transplantation |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a non-Hodgkin’s lymphoma |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Gregorek, Hanna |e verfasserin |4 aut | |
| 700 | 1 | |a Chrzanowska, Krystyna |e verfasserin |4 aut | |
| 700 | 1 | |a Piątosa, Barbara |e verfasserin |4 aut | |
| 700 | 1 | |a Pietrucha, Barbara |e verfasserin |4 aut | |
| 700 | 1 | |a Heropolitańska-Pliszka, Edyta |e verfasserin |4 aut | |
| 700 | 1 | |a Pac, Małgorzata |e verfasserin |4 aut | |
| 700 | 1 | |a Klaudel-Dreszler, Maja |e verfasserin |4 aut | |
| 700 | 1 | |a Kostyuchenko, Larysa |e verfasserin |4 aut | |
| 700 | 1 | |a Pasic, Srdjan |e verfasserin |4 aut | |
| 700 | 1 | |a Marodi, Laszlo |e verfasserin |4 aut | |
| 700 | 1 | |a Belohradsky, Bernd H. |e verfasserin |4 aut | |
| 700 | 1 | |a Čižnár, Peter |e verfasserin |4 aut | |
| 700 | 1 | |a Shcherbina, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Kilic, Sara Sebnem |e verfasserin |4 aut | |
| 700 | 1 | |a Baumann, Ulrich |e verfasserin |4 aut | |
| 700 | 1 | |a Seidel, Markus G. |e verfasserin |4 aut | |
| 700 | 1 | |a Gennery, Andrew R. |e verfasserin |4 aut | |
| 700 | 1 | |a Syczewska, Małgorzata |e verfasserin |4 aut | |
| 700 | 1 | |a Mikołuć, Bożena |e verfasserin |4 aut | |
| 700 | 1 | |a Kałwak, Krzysztof |e verfasserin |4 aut | |
| 700 | 1 | |a Styczyński, Jan |e verfasserin |4 aut | |
| 700 | 1 | |a Pieczonka, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Drabko, Katarzyna |e verfasserin |4 aut | |
| 700 | 1 | |a Wakulińska, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Gathmann, Benjamin |e verfasserin |4 aut | |
| 700 | 1 | |a Albert, Michael H. |e verfasserin |4 aut | |
| 700 | 1 | |a Skarżyńska, Urszula |e verfasserin |4 aut | |
| 700 | 1 | |a Bernatowska, Ewa |e verfasserin |4 aut | |
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10.1007/s10875-015-0186-9 doi (DE-627)SPR014244950 (SPR)s10875-015-0186-9-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Wolska-Kuśnierz, Beata verfasserin aut Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis 2015 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. Nijmegen breakage syndrome (dpeaa)DE-He213 primary immunodeficiencies (dpeaa)DE-He213 chromosomal instability (dpeaa)DE-He213 hematopoietic stem cell transplantation (dpeaa)DE-He213 non-Hodgkin’s lymphoma (dpeaa)DE-He213 Gregorek, Hanna verfasserin aut Chrzanowska, Krystyna verfasserin aut Piątosa, Barbara verfasserin aut Pietrucha, Barbara verfasserin aut Heropolitańska-Pliszka, Edyta verfasserin aut Pac, Małgorzata verfasserin aut Klaudel-Dreszler, Maja verfasserin aut Kostyuchenko, Larysa verfasserin aut Pasic, Srdjan verfasserin aut Marodi, Laszlo verfasserin aut Belohradsky, Bernd H. verfasserin aut Čižnár, Peter verfasserin aut Shcherbina, Anna verfasserin aut Kilic, Sara Sebnem verfasserin aut Baumann, Ulrich verfasserin aut Seidel, Markus G. verfasserin aut Gennery, Andrew R. verfasserin aut Syczewska, Małgorzata verfasserin aut Mikołuć, Bożena verfasserin aut Kałwak, Krzysztof verfasserin aut Styczyński, Jan verfasserin aut Pieczonka, Anna verfasserin aut Drabko, Katarzyna verfasserin aut Wakulińska, Anna verfasserin aut Gathmann, Benjamin verfasserin aut Albert, Michael H. verfasserin aut Skarżyńska, Urszula verfasserin aut Bernatowska, Ewa verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 35(2015), 6 vom: 01. Aug., Seite 538-549 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:35 year:2015 number:6 day:01 month:08 pages:538-549 https://dx.doi.org/10.1007/s10875-015-0186-9 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 35 2015 6 01 08 538-549 |
| spelling |
10.1007/s10875-015-0186-9 doi (DE-627)SPR014244950 (SPR)s10875-015-0186-9-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Wolska-Kuśnierz, Beata verfasserin aut Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis 2015 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. Nijmegen breakage syndrome (dpeaa)DE-He213 primary immunodeficiencies (dpeaa)DE-He213 chromosomal instability (dpeaa)DE-He213 hematopoietic stem cell transplantation (dpeaa)DE-He213 non-Hodgkin’s lymphoma (dpeaa)DE-He213 Gregorek, Hanna verfasserin aut Chrzanowska, Krystyna verfasserin aut Piątosa, Barbara verfasserin aut Pietrucha, Barbara verfasserin aut Heropolitańska-Pliszka, Edyta verfasserin aut Pac, Małgorzata verfasserin aut Klaudel-Dreszler, Maja verfasserin aut Kostyuchenko, Larysa verfasserin aut Pasic, Srdjan verfasserin aut Marodi, Laszlo verfasserin aut Belohradsky, Bernd H. verfasserin aut Čižnár, Peter verfasserin aut Shcherbina, Anna verfasserin aut Kilic, Sara Sebnem verfasserin aut Baumann, Ulrich verfasserin aut Seidel, Markus G. verfasserin aut Gennery, Andrew R. verfasserin aut Syczewska, Małgorzata verfasserin aut Mikołuć, Bożena verfasserin aut Kałwak, Krzysztof verfasserin aut Styczyński, Jan verfasserin aut Pieczonka, Anna verfasserin aut Drabko, Katarzyna verfasserin aut Wakulińska, Anna verfasserin aut Gathmann, Benjamin verfasserin aut Albert, Michael H. verfasserin aut Skarżyńska, Urszula verfasserin aut Bernatowska, Ewa verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 35(2015), 6 vom: 01. Aug., Seite 538-549 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:35 year:2015 number:6 day:01 month:08 pages:538-549 https://dx.doi.org/10.1007/s10875-015-0186-9 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 35 2015 6 01 08 538-549 |
| allfields_unstemmed |
10.1007/s10875-015-0186-9 doi (DE-627)SPR014244950 (SPR)s10875-015-0186-9-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Wolska-Kuśnierz, Beata verfasserin aut Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis 2015 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. Nijmegen breakage syndrome (dpeaa)DE-He213 primary immunodeficiencies (dpeaa)DE-He213 chromosomal instability (dpeaa)DE-He213 hematopoietic stem cell transplantation (dpeaa)DE-He213 non-Hodgkin’s lymphoma (dpeaa)DE-He213 Gregorek, Hanna verfasserin aut Chrzanowska, Krystyna verfasserin aut Piątosa, Barbara verfasserin aut Pietrucha, Barbara verfasserin aut Heropolitańska-Pliszka, Edyta verfasserin aut Pac, Małgorzata verfasserin aut Klaudel-Dreszler, Maja verfasserin aut Kostyuchenko, Larysa verfasserin aut Pasic, Srdjan verfasserin aut Marodi, Laszlo verfasserin aut Belohradsky, Bernd H. verfasserin aut Čižnár, Peter verfasserin aut Shcherbina, Anna verfasserin aut Kilic, Sara Sebnem verfasserin aut Baumann, Ulrich verfasserin aut Seidel, Markus G. verfasserin aut Gennery, Andrew R. verfasserin aut Syczewska, Małgorzata verfasserin aut Mikołuć, Bożena verfasserin aut Kałwak, Krzysztof verfasserin aut Styczyński, Jan verfasserin aut Pieczonka, Anna verfasserin aut Drabko, Katarzyna verfasserin aut Wakulińska, Anna verfasserin aut Gathmann, Benjamin verfasserin aut Albert, Michael H. verfasserin aut Skarżyńska, Urszula verfasserin aut Bernatowska, Ewa verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 35(2015), 6 vom: 01. Aug., Seite 538-549 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:35 year:2015 number:6 day:01 month:08 pages:538-549 https://dx.doi.org/10.1007/s10875-015-0186-9 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 35 2015 6 01 08 538-549 |
| allfieldsGer |
10.1007/s10875-015-0186-9 doi (DE-627)SPR014244950 (SPR)s10875-015-0186-9-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Wolska-Kuśnierz, Beata verfasserin aut Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis 2015 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. Nijmegen breakage syndrome (dpeaa)DE-He213 primary immunodeficiencies (dpeaa)DE-He213 chromosomal instability (dpeaa)DE-He213 hematopoietic stem cell transplantation (dpeaa)DE-He213 non-Hodgkin’s lymphoma (dpeaa)DE-He213 Gregorek, Hanna verfasserin aut Chrzanowska, Krystyna verfasserin aut Piątosa, Barbara verfasserin aut Pietrucha, Barbara verfasserin aut Heropolitańska-Pliszka, Edyta verfasserin aut Pac, Małgorzata verfasserin aut Klaudel-Dreszler, Maja verfasserin aut Kostyuchenko, Larysa verfasserin aut Pasic, Srdjan verfasserin aut Marodi, Laszlo verfasserin aut Belohradsky, Bernd H. verfasserin aut Čižnár, Peter verfasserin aut Shcherbina, Anna verfasserin aut Kilic, Sara Sebnem verfasserin aut Baumann, Ulrich verfasserin aut Seidel, Markus G. verfasserin aut Gennery, Andrew R. verfasserin aut Syczewska, Małgorzata verfasserin aut Mikołuć, Bożena verfasserin aut Kałwak, Krzysztof verfasserin aut Styczyński, Jan verfasserin aut Pieczonka, Anna verfasserin aut Drabko, Katarzyna verfasserin aut Wakulińska, Anna verfasserin aut Gathmann, Benjamin verfasserin aut Albert, Michael H. verfasserin aut Skarżyńska, Urszula verfasserin aut Bernatowska, Ewa verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 35(2015), 6 vom: 01. Aug., Seite 538-549 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:35 year:2015 number:6 day:01 month:08 pages:538-549 https://dx.doi.org/10.1007/s10875-015-0186-9 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 35 2015 6 01 08 538-549 |
| allfieldsSound |
10.1007/s10875-015-0186-9 doi (DE-627)SPR014244950 (SPR)s10875-015-0186-9-e DE-627 ger DE-627 rakwb eng 610 ASE 44.45 bkl Wolska-Kuśnierz, Beata verfasserin aut Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis 2015 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. Nijmegen breakage syndrome (dpeaa)DE-He213 primary immunodeficiencies (dpeaa)DE-He213 chromosomal instability (dpeaa)DE-He213 hematopoietic stem cell transplantation (dpeaa)DE-He213 non-Hodgkin’s lymphoma (dpeaa)DE-He213 Gregorek, Hanna verfasserin aut Chrzanowska, Krystyna verfasserin aut Piątosa, Barbara verfasserin aut Pietrucha, Barbara verfasserin aut Heropolitańska-Pliszka, Edyta verfasserin aut Pac, Małgorzata verfasserin aut Klaudel-Dreszler, Maja verfasserin aut Kostyuchenko, Larysa verfasserin aut Pasic, Srdjan verfasserin aut Marodi, Laszlo verfasserin aut Belohradsky, Bernd H. verfasserin aut Čižnár, Peter verfasserin aut Shcherbina, Anna verfasserin aut Kilic, Sara Sebnem verfasserin aut Baumann, Ulrich verfasserin aut Seidel, Markus G. verfasserin aut Gennery, Andrew R. verfasserin aut Syczewska, Małgorzata verfasserin aut Mikołuć, Bożena verfasserin aut Kałwak, Krzysztof verfasserin aut Styczyński, Jan verfasserin aut Pieczonka, Anna verfasserin aut Drabko, Katarzyna verfasserin aut Wakulińska, Anna verfasserin aut Gathmann, Benjamin verfasserin aut Albert, Michael H. verfasserin aut Skarżyńska, Urszula verfasserin aut Bernatowska, Ewa verfasserin aut Enthalten in Journal of clinical immunology Dordrecht [u.a.] : Springer Science + Business Media B.V, 1981 35(2015), 6 vom: 01. Aug., Seite 538-549 (DE-627)320573362 (DE-600)2016755-6 1573-2592 nnns volume:35 year:2015 number:6 day:01 month:08 pages:538-549 https://dx.doi.org/10.1007/s10875-015-0186-9 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 35 2015 6 01 08 538-549 |
| language |
English |
| source |
Enthalten in Journal of clinical immunology 35(2015), 6 vom: 01. Aug., Seite 538-549 volume:35 year:2015 number:6 day:01 month:08 pages:538-549 |
| sourceStr |
Enthalten in Journal of clinical immunology 35(2015), 6 vom: 01. Aug., Seite 538-549 volume:35 year:2015 number:6 day:01 month:08 pages:538-549 |
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Article |
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findex.gbv.de |
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Nijmegen breakage syndrome primary immunodeficiencies chromosomal instability hematopoietic stem cell transplantation non-Hodgkin’s lymphoma |
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610 |
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Journal of clinical immunology |
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Wolska-Kuśnierz, Beata @@aut@@ Gregorek, Hanna @@aut@@ Chrzanowska, Krystyna @@aut@@ Piątosa, Barbara @@aut@@ Pietrucha, Barbara @@aut@@ Heropolitańska-Pliszka, Edyta @@aut@@ Pac, Małgorzata @@aut@@ Klaudel-Dreszler, Maja @@aut@@ Kostyuchenko, Larysa @@aut@@ Pasic, Srdjan @@aut@@ Marodi, Laszlo @@aut@@ Belohradsky, Bernd H. @@aut@@ Čižnár, Peter @@aut@@ Shcherbina, Anna @@aut@@ Kilic, Sara Sebnem @@aut@@ Baumann, Ulrich @@aut@@ Seidel, Markus G. @@aut@@ Gennery, Andrew R. @@aut@@ Syczewska, Małgorzata @@aut@@ Mikołuć, Bożena @@aut@@ Kałwak, Krzysztof @@aut@@ Styczyński, Jan @@aut@@ Pieczonka, Anna @@aut@@ Drabko, Katarzyna @@aut@@ Wakulińska, Anna @@aut@@ Gathmann, Benjamin @@aut@@ Albert, Michael H. @@aut@@ Skarżyńska, Urszula @@aut@@ Bernatowska, Ewa @@aut@@ |
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2015-08-01T00:00:00Z |
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320573362 |
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3610 |
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SPR014244950 |
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englisch |
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This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. 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610 ASE 44.45 bkl Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis Nijmegen breakage syndrome (dpeaa)DE-He213 primary immunodeficiencies (dpeaa)DE-He213 chromosomal instability (dpeaa)DE-He213 hematopoietic stem cell transplantation (dpeaa)DE-He213 non-Hodgkin’s lymphoma (dpeaa)DE-He213 |
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Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis |
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Wolska-Kuśnierz, Beata Gregorek, Hanna Chrzanowska, Krystyna Piątosa, Barbara Pietrucha, Barbara Heropolitańska-Pliszka, Edyta Pac, Małgorzata Klaudel-Dreszler, Maja Kostyuchenko, Larysa Pasic, Srdjan Marodi, Laszlo Belohradsky, Bernd H. Čižnár, Peter Shcherbina, Anna Kilic, Sara Sebnem Baumann, Ulrich Seidel, Markus G. Gennery, Andrew R. Syczewska, Małgorzata Mikołuć, Bożena Kałwak, Krzysztof Styczyński, Jan Pieczonka, Anna Drabko, Katarzyna Wakulińska, Anna Gathmann, Benjamin Albert, Michael H. Skarżyńska, Urszula Bernatowska, Ewa |
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nijmegen breakage syndrome: clinical and immunological features, long-term outcome and treatment options – a retrospective analysis |
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Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options – a Retrospective Analysis |
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Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. |
| abstractGer |
Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. |
| abstract_unstemmed |
Purpose Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation. Methods The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed. Results Of the 149 NBS patients, 91 (61 %), of median age 14.3 years, remained alive at the time of analysis. These patients were clinically heterogeneous, with variable immune defects, ranging from negligible to severe dysfunction. Humoral deficiencies predisposed NBS patients to recurrent/chronic respiratory tract infections and worsened long-term clinical prognosis. Eighty malignancies, most of lymphoid origin (especially non-Hodgkin’s lymphomas), were diagnosed in 42 % of patients, with malignancy being the leading cause of death in this cohort. Survival probabilities at 5, 10, 20 and 30 years of age were 95, 85, 50 and 35 %, respectively, and were significantly lower in patients with than without malignancies. Conclusions The extremely high incidence of malignancies, mostly non-Hodgkin’s lymphomas, was the main risk factor affecting survival probability in NBS patients. Because treatment of NBS is very difficult and frequently unsuccessful, the search for an alternative medical intervention such as hematopoietic stem cell transplantation is of great clinical importance. |
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Gregorek, Hanna Chrzanowska, Krystyna Piątosa, Barbara Pietrucha, Barbara Heropolitańska-Pliszka, Edyta Pac, Małgorzata Klaudel-Dreszler, Maja Kostyuchenko, Larysa Pasic, Srdjan Marodi, Laszlo Belohradsky, Bernd H. Čižnár, Peter Shcherbina, Anna Kilic, Sara Sebnem Baumann, Ulrich Seidel, Markus G. Gennery, Andrew R. Syczewska, Małgorzata Mikołuć, Bożena Kałwak, Krzysztof Styczyński, Jan Pieczonka, Anna Drabko, Katarzyna Wakulińska, Anna Gathmann, Benjamin Albert, Michael H. Skarżyńska, Urszula Bernatowska, Ewa |
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