title: Down syndrome and acute lymphoblastic leukaemia.
abstract: Acute lymphoblastic leukaemia in children with Down syndrome (ALL-DS) is characterised by unique clinical and biological features. Notable among these are an absence of ALL in DS patients <1 year of age; a lower incidence of favourable and unfavourable chromosomal translocations; heightened sensitivity to methotrexate; and an increased propensity to infections. Although children with ALL-DS have historically fared less well than their non-DS counterparts (ALL-NDS), recent data indicate that outcomes in ALL-DS are now comparable with ALL-NDS with risk-adapted therapies, after adjusting for biological differences between the ALL-DS and ALL-NDS populations. Given the increased risk of ALL-DS patients for treatment-related toxicities, further intensification of therapy may not yield continued improvements in survival. Future investigations in the ALL-DS population should focus on maintaining excellent outcomes while reducing treatment-related complications through novel treatment strategies, such as the integration of targeted noncytotoxic agents.
authors.affiliation: Division of Pediatric Hematology/Oncology, Vanderbilt Children's Hospital and Vanderbilt-Ingram Cancer Center, Nashville, TN 37232, USA. jim.whitlock@vanderbilt.edu
authors.forename: James A
authors.initials: JA
authors.lastname: Whitlock
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D007223:Infant; D008727:Methotrexate / Q000627:therapeutic use; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D008727:Methotrexate