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Description
| - Childhood BCR-ABL1-positive B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has an unfavorable outcome and shows high frequency of IKZF1 deletions. The prognostic value of IKZF1 deletions was evaluated in 2 cohorts of BCR-ABL1-positive BCP-ALL patients, before tyrosine kinase inhibitors (pre-TKI) and after introduction of imatinib (in the European Study for Philadelphia-Acute Lymphoblastic Leukemia [EsPhALL]). In 126/191 (66%) cases an IKZF1 deletion was detected. In the pre-TKI cohort, IKZF1-deleted patients had an unfavorable outcome compared with wild-type patients (4-year disease-free survival [DFS] of 30.0 +/- 6.8% vs 57.5 +/- 9.4%; P = .01). In the EsPhALL cohort, the IKZF1 deletions were associated with an unfavorable prognosis in patients stratified in the good-risk arm based on early clinical response (4-year DFS of 51.9 +/- 8.8% for IKZF1-deleted vs 78.6 +/- 13.9% for IKZF1 wild-type; P = .03), even when treated with imatinib (4-year DFS of 55.5 +/- 9.5% for IKZF1-deleted vs 75.0 +/- 21.7% for IKZF1 wild-type; P = .05). In conclusion, the highly unfavorable outcome for childhood BCR-ABL1-positive BCP-ALL with IKZF1 deletions, irrespective of imatinib exposure, underscores the need for alternative therapies. In contrast, good-risk patients with IKZF1 wild-type responded remarkably well to imatinib-containing regimens, providing a rationale to potentially avoid hematopoietic stem-cell transplantation in this subset of patients.
- Childhood BCR-ABL1-positive B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has an unfavorable outcome and shows high frequency of IKZF1 deletions. The prognostic value of IKZF1 deletions was evaluated in 2 cohorts of BCR-ABL1-positive BCP-ALL patients, before tyrosine kinase inhibitors (pre-TKI) and after introduction of imatinib (in the European Study for Philadelphia-Acute Lymphoblastic Leukemia [EsPhALL]). In 126/191 (66%) cases an IKZF1 deletion was detected. In the pre-TKI cohort, IKZF1-deleted patients had an unfavorable outcome compared with wild-type patients (4-year disease-free survival [DFS] of 30.0 +/- 6.8% vs 57.5 +/- 9.4%; P = .01). In the EsPhALL cohort, the IKZF1 deletions were associated with an unfavorable prognosis in patients stratified in the good-risk arm based on early clinical response (4-year DFS of 51.9 +/- 8.8% for IKZF1-deleted vs 78.6 +/- 13.9% for IKZF1 wild-type; P = .03), even when treated with imatinib (4-year DFS of 55.5 +/- 9.5% for IKZF1-deleted vs 75.0 +/- 21.7% for IKZF1 wild-type; P = .05). In conclusion, the highly unfavorable outcome for childhood BCR-ABL1-positive BCP-ALL with IKZF1 deletions, irrespective of imatinib exposure, underscores the need for alternative therapies. In contrast, good-risk patients with IKZF1 wild-type responded remarkably well to imatinib-containing regimens, providing a rationale to potentially avoid hematopoietic stem-cell transplantation in this subset of patients. (en)
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Title
| - IKZF1 status as a prognostic feature in BCR-ABL1-positive childhood ALL
- IKZF1 status as a prognostic feature in BCR-ABL1-positive childhood ALL (en)
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skos:prefLabel
| - IKZF1 status as a prognostic feature in BCR-ABL1-positive childhood ALL
- IKZF1 status as a prognostic feature in BCR-ABL1-positive childhood ALL (en)
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skos:notation
| - RIV/00216208:11130/14:10292893!RIV15-MSM-11130___
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http://linked.open...avai/riv/aktivita
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http://linked.open...avai/riv/aktivity
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http://linked.open...iv/cisloPeriodika
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http://linked.open...vai/riv/dodaniDat
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http://linked.open...aciTvurceVysledku
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http://linked.open.../riv/druhVysledku
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http://linked.open...iv/duvernostUdaju
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http://linked.open...titaPredkladatele
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http://linked.open...dnocenehoVysledku
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http://linked.open...ai/riv/idVysledku
| - RIV/00216208:11130/14:10292893
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http://linked.open...riv/jazykVysledku
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http://linked.open.../riv/klicovaSlova
| - adolescents; identification; gene; therapy; receptor; ikaros; children; deletions; b-cell precursor; acute lymphoblastic-leukemia (en)
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http://linked.open.../riv/klicoveSlovo
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http://linked.open...odStatuVydavatele
| - US - Spojené státy americké
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http://linked.open...ontrolniKodProRIV
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http://linked.open...i/riv/nazevZdroje
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http://linked.open...in/vavai/riv/obor
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http://linked.open...ichTvurcuVysledku
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http://linked.open...cetTvurcuVysledku
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http://linked.open...vavai/riv/projekt
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http://linked.open...UplatneniVysledku
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http://linked.open...v/svazekPeriodika
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http://linked.open...iv/tvurceVysledku
| - Trka, Jan
- Žaliová, Markéta
- Pieters, Rob
- Schrappe, Martin
- Stanulla, Martin
- Cazzaniga, Giovanni
- Biondi, Andrea
- Cave, Helene
- De Lorenzo, Paola
- Harrison, Christine J.
- Mottadelli, Federica
- Sáha, Vaskar
- Valsecchi, Maria Grazia
- den Boer, Monique L.
- te Kronnie, Gertruuy
- van der Veer, Arian
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http://linked.open...ain/vavai/riv/wos
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issn
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number of pages
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http://bibframe.org/vocab/doi
| - 10.1182/blood-2013-06-509794
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http://localhost/t...ganizacniJednotka
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