Blood, 15 September 2006, Vol. 108, No. 6, pp. 1984-1990.
Prepublished online as a Blood First Edition Paper on May 18, 2006; DOI 10.1182/blood-2006-04-015990.
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Submitted April 11, 2006
Accepted April 29, 2006
Expression of the outcome predictor in acute leukemia 1
(OPAL1) gene is not an independent prognostic factor in
patients treated on COALL or St. Jude protocols
Amy Holleman, Monique L den Boer*, Meyling H Cheok, Karin M Kazemier, Deqing Pei, James R Downing, Gritta E Janka-Schaub, Ulrich Gobel, Ulrike Graubner, Ching-Hon Pui, William E Evans, and Rob Pieters
Pediatric Oncology/Hematology, Erasmus MC/Sophia Children's Hospital
Pharmaceutical Sciences, St. Jude Children's Research Hospital
Biostatistics, St. Jude Children's Research Hospital
Pathology, St. Jude Children's Research Hospital
Hematology and Oncology, University Children's Hospital/ COALL study group
Pediatric Hematology and Oncology,Heinrich-Heine-University/ COALL study group
Pediatric Oncology,Dr. von Haunersches Children's Hospital/ COALL study group
Hematology and Oncology, St. Jude Children's Research Hospital
* Corresponding author; email: m.l.denboer{at}erasmusmc.nl.
New prognostic factors may result in better risk
classification and improved treatment of children with
acute lymphoblastic leukemia (ALL). Recently, high
expression of a gene named OPAL1 (Outcome Predictor in
Acute Leukemia) was reported to be associated with
favorable prognosis in ALL. Therefore, we investigated
whether OPAL1 expression was of prognostic importance in
two independent cohorts of children with ALL treated on
COALL-92/-97 (N=180) and on St. Jude Total 13 protocols
(N=257). We observed a consistently higher (2.8-fold)
expression of OPAL1 in TEL-AML1-positive ALL compared to
TEL-AML1-negative ALL in both cohorts, but higher OPAL1
expression was not consistently associated with other
favorable prognostic indicators such as age and white
blood cell count, or ALL genetic subtype. Lower OPAL1
expression was also not associated with increased in
vitro drug resistance. Multivariate analyses including
known risk factors showed that OPAL1 expression was not
independently related to prognosis in either the COALL
or St. Jude cohorts. In conclusion, OPAL1 expression may
not be an independent prognostic feature in childhood
ALL and its previously reported prognostic impact
appears to be treatment-dependent.
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