About: IKZF1 status as a prognostic feature in BCR-ABL1-positive childhood ALL     Goto   Sponge   NotDistinct   Permalink

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  • 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)
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)
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)
skos:notation
  • RIV/00216208:11130/14:10292893!RIV15-MSM-11130___
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • I, P(GBP302/12/G101)
http://linked.open...iv/cisloPeriodika
  • 11
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  • 20445
http://linked.open...ai/riv/idVysledku
  • RIV/00216208:11130/14:10292893
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • adolescents; identification; gene; therapy; receptor; ikaros; children; deletions; b-cell precursor; acute lymphoblastic-leukemia (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • US - Spojené státy americké
http://linked.open...ontrolniKodProRIV
  • [9F72C7C8A5F4]
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  • Blood
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http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 123
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
http://linked.open...ain/vavai/riv/wos
  • 000335847700016
issn
  • 0006-4971
number of pages
http://bibframe.org/vocab/doi
  • 10.1182/blood-2013-06-509794
http://localhost/t...ganizacniJednotka
  • 11130
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