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Irish Journal of Medical Science (1971 -)

, Volume 184, Issue 4, pp 877–882 | Cite as

Child and adolescent Down syndrome-associated leukaemia: the Irish experience

  • C. O’Rafferty
  • J. Kelly
  • L. Storey
  • C. Ryan
  • A. O’Marcaigh
  • O. Smith
Original Article

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.

Aims

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.

Conclusion

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.

Keywords

Down syndrome Acute lymphoblastic leukaemia Down syndrome myeloid leukaemia Megakaryoblastic leukaemia Treatment-related mortality Prognosis 

Notes

Conflict of interest

None.

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Copyright information

© Royal Academy of Medicine in Ireland 2014

Authors and Affiliations

  • C. O’Rafferty
    • 1
  • J. Kelly
    • 2
  • L. Storey
    • 1
  • C. Ryan
    • 3
  • A. O’Marcaigh
    • 1
  • O. Smith
    • 1
    • 4
  1. 1.Department of HaematologyOur Lady’s Children’s HospitalDublin 12Ireland
  2. 2.National Centre for Medical GeneticsOur Lady’s Children’s HospitalDublin 12Ireland
  3. 3.Department of HaematologyMercy HospitalCorkIreland
  4. 4.Trinity CollegeDublin 2Ireland

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