Outcome of Down syndrome associated acute lymphoblastic leukaemia treated on a contemporary protocol

Br J Haematol. 2014 May;165(4):552-5. doi: 10.1111/bjh.12739. Epub 2014 Jan 15.

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.

Keywords: Down syndrome; lymphoblastic leukaemia; treatment.

Publication types

  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Child
  • Child, Preschool
  • Chromosome Aberrations
  • Disease-Free Survival
  • Down Syndrome / complications*
  • Female
  • Follow-Up Studies
  • Gastrointestinal Hemorrhage / etiology
  • Gastrointestinal Hemorrhage / mortality
  • Genetic Predisposition to Disease
  • Humans
  • Infant
  • Infections / etiology
  • Infections / mortality
  • Male
  • Neoplasm, Residual
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • Recurrence
  • Risk Assessment
  • Treatment Outcome
  • Young Adult