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Nijmegen breakage syndrome (NBS) as a risk factor for CNS involvement in childhood acute lymphoblastic leukemia

Authors

  • Agata Pastorczak MD,

    Corresponding author
    1. Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Lodz, Poland
    • Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Street 36/50, Lodz 91-738, Poland.
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  • Malgorzata Stolarska MD, PhD,

    1. Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Lodz, Poland
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  • Joanna Trelińska MD, PhD,

    1. Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Lodz, Poland
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  • Joanna Zawitkowska MD, PhD,

    1. Department of Pediatric Hematology and Oncology, Medical University of Lublin, Lublin, Poland
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  • Jerzy Kowalczyk MD, PhD,

    1. Department of Pediatric Hematology and Oncology, Medical University of Lublin, Lublin, Poland
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  • Wojciech Mlynarski MD, PhD,

    1. Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Lodz, Poland
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  • on behalf of the Polish Pediatric Leukemia/Lymphoma Study Group


  • Conflict of interest: Nothing to declare.

Abstract

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.

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