Demographic, clinical, and outcome features of children with acute lymphoblastic leukemia and CRLF2 deregulation: results from the MRC ALL97 clinical trial

Hannah M. Ensor, Claire Schwab, Lisa J. Russell, Sue M. Richards, Heather Morrison, Dino Masic, Lisa Jones, Sally E. Kinsey, Ajay J. Vora, Christopher D. Mitchell, Christine J. Harrison, Anthony V. Moorman

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

Deregulated expression of CRLF2 (CRLF2-d) arises via its juxtaposition to the IGH@ enhancer or P2RY8 promoter. Among 865 BCP-ALL children treated on MRC ALL97, 52 (6%) has CRLF2-d but it was more prevalent among Down syndrome patients (54%). P2RY8-CRLF2 (n=43) was more frequent than IGH@-CRLF2 (n=9). CRLF2-d was not associated with age, sex or white cell count, but IGH@- CRLF2 patients were older than P2RY8-CRLF2 patients (median 8 v 4 years, p=0.0017). Patients with CRLF2-d were more likely to present with enlarged livers and spleens (38% v 18%, p<0.001). CRLF2-d was not seen in conjunction with established chromosomal translocations but 6 (12%) cases had high hyperdiploidy and 5 (10%) iAMP21. Univariate analysis suggested that CRLF2-d was associated with an inferior outcome: [EFS HR=2.27 (95% CI 1.48-3.47), p<0.001, OS 3.69 (2.34-5.84), p<0.001]. However, multivariate analysis indicated that its effect was mediated by other risk factors such as cytogenetics and DS status [EFS 1.45 (0.88-2.39), p=0.140, OS 1.90 (1.08-3.36), p=0.027]. Although the outcome of IGH@-CRLF2 patients appeared inferior compared to P2RY8-CRLF2 patients the result was not significant [EFS 2.69 (1.15-6.31), p=0.023; OS 2.86 (1.15-6.79), p=0.021]. Therefore, we concluded that patients with CRLF2-d should be classified into the intermediate cytogenetic risk group.
Original languageEnglish
Pages (from-to)2129-2136
JournalBlood
Volume117
Issue number7
Early online date24 Nov 2010
DOIs
Publication statusE-pub ahead of print - 24 Nov 2010

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