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DOI: 10.1055/s-0031-1277064
IKZF1 deletion is an independent predictor of outcome in pediatric acute lymphoblastic leukemia treated according to the ALL-BFM 2000 protocol
Introduction: Alteration of the IKZF1 gene has been associated with a poor outcome in pediatric precursor B-cell acute lymphoblastic leukemia (ALL).
Method: To assess the prognostic value of IKZF1 deletions we screened a representative cohort of 409 pediatric ALL patients treated on the German ALL-BFM 2000 study protocol, by multiplex ligation-dependent probe amplification (MLPA).
Results: Patients with IKZF1 deletion had a significantly lower 5-year event-free survival (EFS) compared to not-deleted patients, due to a higher cumulative incidence of relapses. IKZF1 deletion conferred a risk of 2.4 for an event when compared to not-deleted patients. IKZF1 deletions were also significantly associated with the P2RY8-CRLF2 rearrangement, but the prognostic value of IKZF1 deletions remained valid independent from this association.
Conclusions: We conclude that IKZF1 deletion is an independent predictor of treatment outcome for patients enrolled on our protocol and represents a candidate marker to be integrated in future algorithms for early risk stratification in pediatric ALL.