Blood, 15 November 2006, Vol. 108, No. 10, pp. 3520-3529.
Prepublished online as a Blood First Edition Paper on July 27, 2006; DOI 10.1182/blood-2006-04-019927.
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Submitted December 28, 2005
Accepted June 30, 2006
The cryptic chromosomal deletion, del(11)(p12p13), as a
new activation mechanism of LMO2 in pediatric T-
cell acute lymphoblastic leukemia
Pieter Van Vlierberghe, Martine van Grotel, H Berna Beverloo, Charles Lee, Tryggvi Helgason, Jessica Buijs-Gladdines, Monique Passier, Elisabeth R van Wering, Anjo J P Veerman, Willem A Kamps, Jules P.P. Meijerink*, and Rob Pieters
Department of Pediatric Oncology/Hematology, Erasmus MC / Sophia Childrens Hospital
Department of Clinical Genetics, Erasmus MC, Rotterdam, The Netherlands
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston
Dutch Childhood Oncology Group (DCOG), The Hague, The Netherlands
BDutch Childhood Oncology Group (DCOG), The Hague, The Netherlands
* Corresponding author; email: j.meijerink{at}erasmusmc.nl.
Abstract
To identify new cytogenetic abnormalities associated
with leukemogenesis or disease outcome, T-ALL patient
samples were analyzed by means of the array-comparative
genome hybridization technique (array-CGH). Here, we
report the identification of a new recurrent and cryptic
deletion on chromosome 11, i.e. the del(11)(p12p13), in
about 4% (6/138) of pediatric T-ALL patients. Detailed
molecular-cytogenetic analysis revealed that this
deletion activates the LMO2 oncogene in 4 out of
6 del(11)(p12p13) positive T-ALL patients, alike
patients with an LMO2 translocation (9/138). The
LMO2 activation mechanism of this deletion is
loss of a negative regulatory region upstream of
LMO2, causing activation of the proximal
LMO2 promoter. LMO2 rearrangements,
including this del(11)(p12p13) and t(11;14)(p13;q11) or t
(7;11)(q35;p13), were found in the absence of other
recurrent cytogenetic abnormalities involving
HOX11L2, HOX11, CALM-AF10,
TAL1, MLL or MYC. LMO2
abnormalities, represent about 9% (13/138) of pediatric
T-ALL cases, and are more frequent in pediatric T-ALL
than appreciated up till now.
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