Abstract
Two boys with neurofibromatosis-Noonan syndrome in whom acute lymphoblastic leukemia (ALL) developed are described. Both patients demonstrated B-lineage leukemias with normal cytogenetics. They were treated with combination chemotherapy and remain in remission off therapy. Patients with neurofibromatosis-Noonan syndrome may be at increased risk for ALL.
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Child, Preschool
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Chromosomes, Human, Pair 12 / genetics
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Chromosomes, Human, Pair 17 / genetics
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Genes, Neurofibromatosis 1
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Genes, ras
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Genetic Predisposition to Disease
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Humans
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Male
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Neurofibromatoses / complications*
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Neurofibromatoses / genetics
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Neurofibromin 1
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Noonan Syndrome / complications*
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Noonan Syndrome / genetics
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Phenotype
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / etiology*
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Proteins / physiology
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Remission Induction
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Risk