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.
Copyright © 2011 Wiley-Liss, Inc.
Publication types
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Multicenter Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Animals
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Central Nervous System / metabolism
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Central Nervous System / pathology*
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Child
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Child, Preschool
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DNA-Binding Proteins / genetics
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DNA-Binding Proteins / metabolism
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Disease-Free Survival
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Female
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Humans
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Infant
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Leukemic Infiltration / drug therapy
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Leukemic Infiltration / metabolism
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Leukemic Infiltration / mortality*
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Leukemic Infiltration / pathology*
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Male
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Mice
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Mice, Knockout
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Nijmegen Breakage Syndrome / drug therapy
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Nijmegen Breakage Syndrome / genetics
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Nijmegen Breakage Syndrome / mortality*
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Nijmegen Breakage Syndrome / pathology*
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Nuclear Proteins / genetics
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Nuclear Proteins / metabolism
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / mortality*
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology*
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Retrospective Studies
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Risk Factors
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Survival Rate
Substances
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DNA-Binding Proteins
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Nuclear Proteins
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RAG2 protein, human
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Rag2 protein, mouse