Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival
Systemic light-chain amyloidosis (AL) is an acquired protein misfolding disease characterized by deposition of immunoglobulin light-chain fibrils most often secreted from clonal plasma cells. In this retrospective study we analyzed the impact of iFISH aberrations on clinical characteristics and outc...
Gespeichert in:
| Hauptverfasser: | , , , , , , , , , , , , , |
|---|---|
| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
22 March 2025
|
| In: |
Annals of hematology
Year: 2025, Jahrgang: 104, Heft: 3, Pages: 1777-1788 |
| ISSN: | 1432-0584 |
| DOI: | 10.1007/s00277-025-06256-7 |
| Online-Zugang: | Verlag, kostenfrei, Volltext: https://doi.org/10.1007/s00277-025-06256-7
|
| Verfasserangaben: | Sara Oubari, Maria Papathanasiou, Lars Michel, Tienush Rassaf, Andreas Thimm, Tim Hagenacker, Daniela Ehling, Stefan Wieczorek, Eyad Naser, Ute Hegenbart, Stefan Schönland, Ulrich Dührsen, Hans Christian Reinhardt, Alexander Carpinteiro |
MARC
| LEADER | 00000caa a2200000 c 4500 | ||
|---|---|---|---|
| 001 | 1937762149 | ||
| 003 | DE-627 | ||
| 005 | 20251120193507.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 251006s2025 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1007/s00277-025-06256-7 |2 doi | |
| 035 | |a (DE-627)1937762149 | ||
| 035 | |a (DE-599)KXP1937762149 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rda | ||
| 041 | |a eng | ||
| 084 | |a 33 |2 sdnb | ||
| 100 | 1 | |a Oubari, Sara |e VerfasserIn |0 (DE-588)1282158627 |0 (DE-627)183777482X |4 aut | |
| 245 | 1 | 0 | |a Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival |c Sara Oubari, Maria Papathanasiou, Lars Michel, Tienush Rassaf, Andreas Thimm, Tim Hagenacker, Daniela Ehling, Stefan Wieczorek, Eyad Naser, Ute Hegenbart, Stefan Schönland, Ulrich Dührsen, Hans Christian Reinhardt, Alexander Carpinteiro |
| 246 | 3 | 3 | |a Gain or amplification of one q twentyone in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival |
| 264 | 1 | |c 22 March 2025 | |
| 300 | |b Illustrationen | ||
| 300 | |a 12 | ||
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Online veröffentlicht: 22. März 2025 | ||
| 500 | |a Gesehen am 06.10.2025 | ||
| 520 | |a Systemic light-chain amyloidosis (AL) is an acquired protein misfolding disease characterized by deposition of immunoglobulin light-chain fibrils most often secreted from clonal plasma cells. In this retrospective study we analyzed the impact of iFISH aberrations on clinical characteristics and outcomes in 175 AL patients presented between 2015 and 2024. The most common aberrations were t(11;14) (57%), deletion 13q14 (33%), +1q21 (21%), hyperdiploidy (21%) and deletion 16q23 (17%). Significant elevations in dFLC levels were observed in patients with + 1q21 (median 407 vs. 213 mg/l, p = 0.04) and deletion 16q23 (median 476 vs. 204, p = 0.006). Only + 1q21 was associated with increased levels of cardiac biomarkers NTproBNP (median 9945 vs. 3538 pg/ml, p = 0.002) and hsTnT (median 110 vs. 53 ng/l, p = 0.002). This resulted in an increased proportion of patients with Mayo stage IIIb (53% vs. 26%, p = 0.01). Patients with + 1q21 had more advanced plasma cell disease (p = 0.0004). Our study highlights for the first time + 1q21 as the key aberration associated with advanced cardiac and plasma cell disease. After 17 months of follow-up, overall survival was significantly worse in patients with + 1q21 treated with daratumumab (7.2 months vs. not reached, p = 0.006). Alternative therapeutic approaches such as CAR-T therapies or bispecific antibodies should be further investigated. | ||
| 650 | 4 | |a +1q21 | |
| 650 | 4 | |a AL amyloidosis | |
| 650 | 4 | |a IFISH | |
| 650 | 4 | |a Mayo stage | |
| 700 | 1 | |a Papathanasiou, Maria |e VerfasserIn |4 aut | |
| 700 | 1 | |a Michel, Lars |e VerfasserIn |4 aut | |
| 700 | 1 | |a Rassaf, Tienush |e VerfasserIn |4 aut | |
| 700 | 1 | |a Thimm, Andreas |e VerfasserIn |4 aut | |
| 700 | 1 | |a Hagenacker, Tim |e VerfasserIn |4 aut | |
| 700 | 1 | |a Ehling, Daniela |e VerfasserIn |4 aut | |
| 700 | 1 | |a Wieczorek, Stefan |e VerfasserIn |4 aut | |
| 700 | 1 | |a Naser, Eyad |e VerfasserIn |4 aut | |
| 700 | 1 | |a Hegenbart, Ute |e VerfasserIn |0 (DE-588)1028373708 |0 (DE-627)73062157X |0 (DE-576)35901903X |4 aut | |
| 700 | 1 | |a Schönland, Stefan |d 1969- |e VerfasserIn |0 (DE-588)122405226 |0 (DE-627)705896137 |0 (DE-576)293255792 |4 aut | |
| 700 | 1 | |a Dührsen, Ulrich |e VerfasserIn |4 aut | |
| 700 | 1 | |a Reinhardt, Hans Christian |e VerfasserIn |4 aut | |
| 700 | 1 | |a Carpinteiro, Alexander |e VerfasserIn |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Annals of hematology |d Berlin : Springer, 1991 |g 104(2025), 3, Seite 1777-1788 |h Online-Ressource |w (DE-627)253389852 |w (DE-600)1458429-3 |w (DE-576)072283157 |x 1432-0584 |7 nnas |a Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival |
| 773 | 1 | 8 | |g volume:104 |g year:2025 |g number:3 |g pages:1777-1788 |g extent:12 |a Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival |
| 856 | 4 | 0 | |u https://doi.org/10.1007/s00277-025-06256-7 |x Verlag |x Resolving-System |z kostenfrei |3 Volltext |
| 951 | |a AR | ||
| 992 | |a 20251006 | ||
| 993 | |a Article | ||
| 994 | |a 2025 | ||
| 998 | |g 122405226 |a Schönland, Stefan |m 122405226:Schönland, Stefan |d 910000 |d 910100 |d 50000 |e 910000PS122405226 |e 910100PS122405226 |e 50000PS122405226 |k 0/910000/ |k 1/910000/910100/ |k 0/50000/ |p 11 | ||
| 998 | |g 1028373708 |a Hegenbart, Ute |m 1028373708:Hegenbart, Ute |d 910000 |d 910100 |d 50000 |e 910000PH1028373708 |e 910100PH1028373708 |e 50000PH1028373708 |k 0/910000/ |k 1/910000/910100/ |k 0/50000/ |p 10 | ||
| 999 | |a KXP-PPN1937762149 |e 478123996X | ||
| BIB | |a Y | ||
| SER | |a journal | ||
| JSO | |a {"note":["Online veröffentlicht: 22. März 2025","Gesehen am 06.10.2025"],"origin":[{"dateIssuedKey":"2025","dateIssuedDisp":"22 March 2025"}],"title":[{"title":"Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival","title_sort":"Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival"}],"type":{"bibl":"article-journal","media":"Online-Ressource"},"relHost":[{"part":{"year":"2025","pages":"1777-1788","issue":"3","text":"104(2025), 3, Seite 1777-1788","volume":"104","extent":"12"},"titleAlt":[{"title":"Hematology"}],"language":["eng"],"id":{"eki":["253389852"],"zdb":["1458429-3"],"issn":["1432-0584"]},"recId":"253389852","physDesc":[{"extent":"Online-Ressource"}],"disp":"Gain or amplification of 1q21 in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survivalAnnals of hematology","type":{"media":"Online-Ressource","bibl":"periodical"},"origin":[{"dateIssuedDisp":"1991-","dateIssuedKey":"1991","publisher":"Springer","publisherPlace":"Berlin ; Heidelberg ; New York"}],"pubHistory":["62, 1 (Februrary 1991)-"],"note":["Gesehen am 24.04.2025","Fortsetzung der Druck-Ausgabe","Ungezählte Beil. ab 80.2001: Supplement"],"title":[{"subtitle":"organ of: Gesellschaft für Thrombose- und Hämostaseforschung, DGHO, Deutsche Gesellschaft für Hämatologie und Onkologie e.V., ÖGHO, Österreichische Gesellschaft für Hämatologie und Onkologie","title_sort":"Annals of hematology","title":"Annals of hematology"}]}],"recId":"1937762149","name":{"displayForm":["Sara Oubari, Maria Papathanasiou, Lars Michel, Tienush Rassaf, Andreas Thimm, Tim Hagenacker, Daniela Ehling, Stefan Wieczorek, Eyad Naser, Ute Hegenbart, Stefan Schönland, Ulrich Dührsen, Hans Christian Reinhardt, Alexander Carpinteiro"]},"physDesc":[{"noteIll":"Illustrationen","extent":"12 S."}],"id":{"eki":["1937762149"],"doi":["10.1007/s00277-025-06256-7"]},"person":[{"display":"Oubari, Sara","family":"Oubari","given":"Sara","role":"aut"},{"given":"Maria","role":"aut","display":"Papathanasiou, Maria","family":"Papathanasiou"},{"given":"Lars","role":"aut","family":"Michel","display":"Michel, Lars"},{"role":"aut","given":"Tienush","family":"Rassaf","display":"Rassaf, Tienush"},{"display":"Thimm, Andreas","family":"Thimm","given":"Andreas","role":"aut"},{"family":"Hagenacker","display":"Hagenacker, Tim","given":"Tim","role":"aut"},{"display":"Ehling, Daniela","family":"Ehling","role":"aut","given":"Daniela"},{"given":"Stefan","role":"aut","family":"Wieczorek","display":"Wieczorek, Stefan"},{"family":"Naser","display":"Naser, Eyad","role":"aut","given":"Eyad"},{"display":"Hegenbart, Ute","family":"Hegenbart","given":"Ute","role":"aut"},{"role":"aut","given":"Stefan","family":"Schönland","display":"Schönland, Stefan"},{"role":"aut","given":"Ulrich","display":"Dührsen, Ulrich","family":"Dührsen"},{"given":"Hans Christian","role":"aut","display":"Reinhardt, Hans Christian","family":"Reinhardt"},{"family":"Carpinteiro","display":"Carpinteiro, Alexander","role":"aut","given":"Alexander"}],"language":["eng"],"titleAlt":[{"title":"Gain or amplification of one q twentyone in systemic light chain amyloidosis is associated with advanced Mayo stage, plasma cell disease and worse overall survival"}]} | ||
| SRT | |a OUBARISARAGAINORAMPL2220 | ||