Deletion of IKZF1 and prognosis in acute lymphoblastic leukaemia
Deletion or mutation of the IKZF1 gene marked a subgroup of childhood B-cell acute lymphoblastic leukaemia with a threefold higher risk of relapse, and these cases shared a gene expression signature with Philadelphia-positive leukaemia.
Overview
Genomic study of 221 children with high-risk B-ALL identifying IKZF1 deletions or mutations in 28.6 percent, associated with a hazard ratio of about 3.5 for relapse, poor outcome independent of other factors, and a gene expression profile resembling BCR-ABL1-positive ALL; findings were validated in a second cohort.
- IKZF1 alterations in 28.6 percent of high-risk B-ALL.
- Hazard ratio for relapse about 3.5 with IKZF1 alteration.
IKZF1 status is part of risk stratification in several paediatric ALL protocols and is a hallmark of Ph-like ALL.
- Prognostic effect is attenuated by measurable residual disease-directed therapy and co-occurring favourable lesions.
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