Demographic, clinical, and outcome features of children with acute lymphoblastic leukemia and CRLF2 deregulation: Results from the MRCALL97 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

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%) had 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 vs 4 years, P = .0017). Patients with CRLF2-d were more likely to present with enlarged livers and spleens (38% vs 18%, P < .001). CRLF2-d was not seen in conjunction with established chromosomal translocations but 6 (12%) cases had high hyperdiploidy, and 5 (10%) had iAMP21. Univariate analysis suggested that CRLF2-d was associated with an inferior outcome: (event-free survival [EFS] hazard ratio 2.27 [95% confidence interval 1.48-3.47],P < .001;OS3.69 [2.34-5.84],P < .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 = .140; OS 1.90 [1.08-3.36], P = .027). Although the outcome of IGH@-CRLF2 patients appeared inferior compared with P2RY8-CRLF2 patients, the result was not significant (EFS 2.69 [1.15-6.31],P = .023;OS2.86 [1.15-6.79],P = .021). Therefore, we concluded that patients with CRLF2-d should be classified into the intermediate cytogenetic risk group.

Original languageEnglish (US)
Pages (from-to)2129-2136
Number of pages8
JournalBlood
Volume117
Issue number7
DOIs
StatePublished - Feb 17 2011

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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