Klin Padiatr 2011; 223 - A3
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

P Breithaupt 1, B Meissner 1, M Zimmermann 2, A Möricke 1, A Schrauder 1, J Harbott 3, WD Ludwig 4, R Köhler 5, CR Bartram 5, M Schrappe 1, G Cario 1, M Stanulla 1
  • 1Department of Pediatrics, University Medical Centre Schleswig-Holstein, Kiel
  • 2Department of Pediatric Hematology and Oncology, Hannover Medical School, Hannover
  • 3Department of Pediatric Hematology and Oncology, University Hospital Gießen and Marburg, Gießen
  • 4Department of Hematology, Oncology and Tumor Immunology, HELIOS-Clinic Berlin-Buch, Berlin
  • 5Department of Human Genetics, University of Heidelberg, Heidelberg

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.