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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NEOPLASIA
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
From the Department of Pediatric Oncology/Hematology, Erasmus Medical Center (MC)/Sophia Children's Hospital, Rotterdam, The Netherlands; the Department of Clinical Genetics, Erasmus MC, Rotterdam, The Netherlands; the Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA; the Dutch Childhood Oncology Group (DCOG), The Hague, The Netherlands; the Department of Pediatric Oncology/Hematology, Vrije Universiteit MC, Amsterdam, The Netherlands; and the Department of Pediatric Oncology, Beatrix Children's Hospital, University MC, Groningen, The Netherlands.
To identify new cytogenetic abnormalities associated with leukemogenesis or disease outcome, T-cell acute lymphoblastic leukemia (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 (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 of 6 del(11)(p12p13)-positive T-ALL patients, in the same manner as in 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 until now.
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