title: Child and adolescent Down syndrome-associated leukaemia: the Irish experience.
abstract: BACKGROUND
  Down syndrome (DS), the most common syndromic chromosomal abnormality is associated with a unique susceptibility to develop both acute myeloid (ML) and lymphoblastic leukaemia (ALL). These leukaemias differ from the non-DS-related types of leukaemia and are thought to be distinct biological entities.
  
  
  OBJECTIVE
  To perform a retrospective review of our experience of treating DS-related leukaemia at Our Lady's Children's Hospital.
  
  
  METHODS
  Data were extracted from a database established in 2000 to prospectively gather data on DS-associated leukaemias and their outcomes following polychemotherapy. Kaplan-Meier survival curves were constructed.
  
  
  RESULTS
  Nineteen patients with DS-ML were treated and 19 with DS-ALL. Sixteen (84%) patients with DS-ML are alive and in complete remission with a median follow-up of 7 years. All deaths in this cohort were due to treatment-related mortality (TRM). Of the DS-ALL patients, 12 (63%) remain alive with a median follow-up of 3.6 years. TRM accounted for five of the six deaths. One death was due to leukaemic relapse.
  
  
  CONCLUSIONS
  High cure rates are seen in DS-ML using contemporary polychemotherapy protocols, however, there is significant TRM in this cohort. DS-ALL does not have the same high cure rate as non-DS-ALL (>90%) and again this is mainly due to an excess of TRM.
authors.affiliation: Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. orafferc@gmail.com.
authors.forename: C
authors.initials: C
authors.lastname: O'Rafferty
authors.affiliation: National Centre for Medical Genetics, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. Johanna.kelly@olchc.ie.
authors.forename: J
authors.initials: J
authors.lastname: Kelly
authors.affiliation: Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. lorna.storey@gmail.com.
authors.forename: L
authors.initials: L
authors.lastname: Storey
authors.affiliation: Department of Haematology, Mercy Hospital, Cork, Ireland. Clryan@muh.ie.
authors.forename: C
authors.initials: C
authors.lastname: Ryan
authors.affiliation: Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. aengus.omarcaigh@olchc.ie.
authors.forename: A
authors.initials: A
authors.lastname: O'Marcaigh
authors.affiliation: Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. owen.smith@olchc.ie.|Trinity College, Dublin 2, Ireland. owen.smith@olchc.ie.
authors.forename: O
authors.initials: O
authors.lastname: Smith
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D015470:Leukemia, Myeloid, Acute / Q000453:epidemiology*; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000453:epidemiology*; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies
keywords: Acute lymphoblastic leukaemia; Down syndrome; Down syndrome myeloid leukaemia; Megakaryoblastic leukaemia; Prognosis; Treatment-related mortality