Deletions in IKZF1 are found in ∼15% of children with B-cell precursor acute lymphoblastic leukemia (BCP-ALL). There is strong evidence for the poor prognosis of IKZF1 deletions affecting exons 4-7 and exons 1-8, but evidence for the remaining 33% of cases harboring other variants of IKZF1 deletions is lacking. In an international multicenter study we analyzed the prognostic value of these rare variants in a case-control design. Each IKZF1-deleted case was matched to three IKZF1 wild-type controls based on cytogenetic subtype, treatment protocol, risk stratification arm, white blood cell count and age. Hazard ratios for the prognostic impact of rare IKZF1 deletions on event-free survival were calculated by matched pair Cox regression. Matched pair analysis for all 134 cases with rare IKZF1 deletions together revealed a poor prognosis (P<0.001) that was evident in each risk stratification arm. Rare variant types with the most unfavorable event-free survival were DEL 2-7 (P=0.03), DEL 2-8 (P=0.002) and DEL-Other (P<0.001). The prognosis of each type of rare variant was equal or worse compared with the well-known major DEL 4-7 and DEL 1-8 IKZF1 deletion variants. We therefore conclude that all variants of rare IKZF1 deletions are associated with an unfavorable prognosis in pediatric BCP-ALL.
Boer J.M., Van Der Veer A., Rizopoulos D., Fiocco M., Sonneveld E., De Groot-Kruseman H.A., et al. (2016). Prognostic value of rare IKZF1 deletion in childhood B-cell precursor acute lymphoblastic leukemia: An international collaborative study. LEUKEMIA, 30(1), 32-38 [10.1038/leu.2015.199].
Citazione: | Boer J.M., Van Der Veer A., Rizopoulos D., Fiocco M., Sonneveld E., De Groot-Kruseman H.A., et al. (2016). Prognostic value of rare IKZF1 deletion in childhood B-cell precursor acute lymphoblastic leukemia: An international collaborative study. LEUKEMIA, 30(1), 32-38 [10.1038/leu.2015.199]. | |
Tipo: | Articolo in rivista - Articolo scientifico | |
Carattere della pubblicazione: | Scientifica | |
Presenza di un coautore afferente ad Istituzioni straniere: | Si | |
Titolo: | Prognostic value of rare IKZF1 deletion in childhood B-cell precursor acute lymphoblastic leukemia: An international collaborative study | |
Autori: | Boer J.M.; Van Der Veer A.; Rizopoulos D.; Fiocco M.; Sonneveld E.; De Groot-Kruseman H.A.; Kuiper R.P.; Hoogerbrugge P.; Horstmann M.; Zaliova M.; Palmi C.; Trka J.; Fronkova E.; Emerenciano M.; Do Socorro Pombo-De-Oliveira M.; Mlynarski W.; Szczepanski T.; Nebral K.; Attarbaschi A.; Venn N.; Sutton R.; Schwab C.J.; Enshaei A.; Vora A.; Stanulla M.; Schrappe M.; Cazzaniga G.; Conter V.; Zimmermann M.; Moorman A.V.; Pieters R.; Den Boer M.L. | |
Autori: | ||
Data di pubblicazione: | 2016 | |
Lingua: | English | |
Rivista: | LEUKEMIA | |
Digital Object Identifier (DOI): | http://dx.doi.org/10.1038/leu.2015.199 | |
Appare nelle tipologie: | 01 - Articolo su rivista |