title: Outcome of Down syndrome associated acute lymphoblastic leukaemia treated on a contemporary protocol.
abstract: We report the outcome for children and young people with Down syndrome-associated acute lymphoblastic leukaemia (DS-ALL) treated on a contemporary protocol. Compared with non-DS ALL, patients with DS-ALL had an inferior event-free survival (65·6% vs. 87·7% at 5 years; P < 0·00005) and overall survival (70·0% vs. 92·2%; P < 0·00005). Excess treatment-related mortality - was primarily responsible for the worse outcomes for DS-ALL (21·6% at 5 years, vs. 3·3%, P < 0·00005). Minimal residual disease (MRD) risk status was highly discriminant for relapse in DS patients with 0/28 relapses in the MRD low risk group.
authors.affiliation: Sheffield Children's Hospital, Sheffield, UK.
authors.forename: Katharine
authors.initials: K
authors.lastname: Patrick
authors.forename: Rachel
authors.initials: R
authors.lastname: Wade
authors.forename: Nicholas
authors.initials: N
authors.lastname: Goulden
authors.forename: Clare
authors.initials: C
authors.lastname: Rowntree
authors.forename: Rachael
authors.initials: R
authors.lastname: Hough
authors.forename: Anthony V
authors.initials: AV
authors.lastname: Moorman
authors.forename: Christopher D
authors.initials: CD
authors.lastname: Mitchell
authors.forename: Ajay
authors.initials: A
authors.lastname: Vora
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D018572:Disease-Free Survival; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006471:Gastrointestinal Hemorrhage / Q000209:etiology / Q000401:mortality; D020022:Genetic Predisposition to Disease; D006801:Humans; D007223:Infant; D007239:Infections / Q000209:etiology / Q000401:mortality; D008297:Male; D018365:Neoplasm, Residual; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000235:genetics; D012008:Recurrence; D018570:Risk Assessment; D016896:Treatment Outcome; D055815:Young Adult
keywords: Down syndrome; lymphoblastic leukaemia; treatment