Microsatellite instability in childhood t cell acute lymphoblastic leukemia

ABSTRACT Acute lymphoblastic leukemia (ALL) is the most frequent cancer encountered in children. Little is known about the molecular pathology of childhood T cell ALL. Oncogenesis is a

multistep process that involves alterations in proto-oncogenes and tumor suppressor genes. Recently, a mutator phenotype detectable by microsatellite instabilities was shown to be associated

with predisposition to cancer. This new mechanism for human carcinogenesis is caused by defects in the DNA replication/repair system. To study the involvement of some of these mutational

events in the development of T cell ALL, we have initiated a systematic search for losses of heterozygosity (LOH) and microsatellite instabilities in children affected with this disease.

These patients were allelotyped by PCR using 56 microsatellite markers located near known or putative tumor suppressor genes. The microsatellite patterns were altered in more than 80% of the

patients. LOH were detected in chromosomes 6p, 12p and 9p. Two third of the patients were deleted for chromosome 9p21, suggesting the involvement of a tumor suppressor gene, probably the

p16 gene. The only patient refractory to chemotherapy was shown to be associated with a mutator phenotype. This is the first documented case of a childhood neoplasia associated with genomic

instabilities. Our results suggest that defects in DNA replication/repair components are involved in the development of a subset of childhood T cell ALL. Access through your institution Buy

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OTHERS THE GENOMIC BASIS OF CHILDHOOD T-LINEAGE ACUTE LYMPHOBLASTIC LEUKAEMIA Article 14 August 2024 INHERENT GENOME INSTABILITY UNDERLIES TRISOMY 21-ASSOCIATED MYELOID MALIGNANCIES Article

20 January 2024 THE GENOMIC LANDSCAPE OF PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA Article 01 September 2022 AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Division of Hematology-Oncology,

Centre de Cancérologie Charles Bruneau, Canada A Baccichet, N Benachenhou, F Couture, J-M Leclerc & D Sinnett * Research Center, Sainte-Justine Hospital, Canada A Baccichet, N

Benachenhou & D Sinnett * Department of Pediatrics, University of Montreal, Canada J-M Leclerc & D Sinnett Authors * A Baccichet View author publications You can also search for this

author inPubMed Google Scholar * N Benachenhou View author publications You can also search for this author inPubMed Google Scholar * F Couture View author publications You can also search

for this author inPubMed Google Scholar * J-M Leclerc View author publications You can also search for this author inPubMed Google Scholar * D Sinnett View author publications You can also

search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Baccichet, A., Benachenhou, N., Couture, F. _et al._

Microsatellite instability in childhood T cell acute lymphoblastic leukemia. _Leukemia_ 11, 797–802 (1997). https://doi.org/10.1038/sj.leu.2400650 Download citation * Received: 05 September

1996 * Accepted: 11 February 1997 * Issue Date: 01 June 1997 * DOI: https://doi.org/10.1038/sj.leu.2400650 SHARE THIS ARTICLE Anyone you share the following link with will be able to read

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KEYWORDS * mutator phenotype * microsatellite instability * mismatch repair loci, childhood T cell leukemia * relapse