C O'Rafferty1, J Kelly2, L Storey3, C Ryan4, A O'Marcaigh5, O Smith6,7. 1. Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. orafferc@gmail.com. 2. National Centre for Medical Genetics, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. Johanna.kelly@olchc.ie. 3. Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. lorna.storey@gmail.com. 4. Department of Haematology, Mercy Hospital, Cork, Ireland. Clryan@muh.ie. 5. Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. aengus.omarcaigh@olchc.ie. 6. Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland. owen.smith@olchc.ie. 7. Trinity College, Dublin 2, Ireland. owen.smith@olchc.ie.
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.
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.
Entities:
Keywords:
Acute lymphoblastic leukaemia; Down syndrome; Down syndrome myeloid leukaemia; Megakaryoblastic leukaemia; Prognosis; Treatment-related mortality
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