Conflict of interest: Nothing to declare.
Nijmegen breakage syndrome (NBS) as a risk factor for CNS involvement in childhood acute lymphoblastic leukemia†
Article first published online: 8 MAR 2011
Copyright © 2011 Wiley-Liss, Inc.
Pediatric Blood & Cancer
Volume 57, Issue 1, pages 160–162, 15 July 2011
How to Cite
Pastorczak, A., Stolarska, M., Trelińska, J., Zawitkowska, J., Kowalczyk, J., Mlynarski, W. and on behalf of the Polish Pediatric Leukemia/Lymphoma Study Group (2011), Nijmegen breakage syndrome (NBS) as a risk factor for CNS involvement in childhood acute lymphoblastic leukemia. Pediatr. Blood Cancer, 57: 160–162. doi: 10.1002/pbc.23073
- Issue published online: 9 MAY 2011
- Article first published online: 8 MAR 2011
- Manuscript Accepted: 13 JAN 2011
- Manuscript Received: 28 JUL 2010
- CNS involvement;
- Nijmegen breakage syndrome
Central nervous system (CNS) involvement is an independent risk factor for poor event-free survival and relapse confined to the CNS. Knock-out mice deprived of RAG2, the protein involved in DNA repair, developed leukemic infiltration within leptomeninges. Therefore, we hypothesized that DNA repair deficiencies in humans, such as Nijmegen breakage syndrome (NBS), may constitute a risk factor for CNS dissemination of acute lymphoblastic leukemia (ALL). Having analyzed the incidence of CNS2/CNS3 status at diagnosis of ALL in two independent cohorts from the Polish Pediatric Leukemia/Lymphoma Study Group, we noticed that among children with NBS CNS involvement was significantly frequent. Pediatr Blood Cancer 2011;57:160–162. © 2011 Wiley-Liss, Inc.