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Down Syndrome and Acute Myeloid Leukemia: An Unique Genetic Sensitivity to Chemotherapy

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Molecularly Targeted Therapy for Childhood Cancer

Abstract

Following the first description of leukemia in a Down syndrome (DS) child in 1930 (Brewster and Cannon 1930), a national survey in 1957 confirmed that DS individuals had an increased risk of developing leukemia (Krivit and Good 1957). It has been estimated that DS children have a 10 to 20-fold increased risk of developing both acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) compared to nonDS children (Taub 2001). A Danish population-based study reported that the cumulative risk of developing leukemia in DS children by the age of 5 was 2.1%, and there was an approximately fourfold higher standardized incidence ratio of AML compared to ALL (Hasle et al. 2000). The Nordic Society of Pediatric Hematology and Oncology (NOPHO) cooperative group reported that DS children with ALL and AML comprised 2.1% and 14%, respectively, of total childhood leukemia cases (Zeller et al. 2005). In the Children’s Cancer Group (CCG) 2891 study, 15% of the AML patients had DS, indicating that DS children comprise one of the largest subgroup of AML patients.

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Acknowledgments

Supported by grants RO1 CA92308 and CA120772 from the National Cancer Institute, the Leukemia and Lymphoma Society, The Elana Fund, The Ring Screw Textron Chair in Pediatric Cancer Research and The Georgie Ginopolis Chair for Pediatric Cancer and Hematology.

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Taub, J.W., Ge, Y., Ravindranath, Y. (2010). Down Syndrome and Acute Myeloid Leukemia: An Unique Genetic Sensitivity to Chemotherapy. In: Houghton, P., Arceci, R. (eds) Molecularly Targeted Therapy for Childhood Cancer. Springer, New York, NY. https://doi.org/10.1007/978-0-387-69062-9_6

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