captures all cases of AML, increasing the reliability of population-based data as case ascertainment and follow-up follow a uniform approach. Aims: We sought to describe the clinical characteristics, incidende and relative survival for patients with AML in the province of Manitoba, with attention to temporal trends and outcomes in rural patients, where access to care may be challenging. Methods: We collected data on consecutively diagnosed adult cases of AML that were reported to the Manitoba Cancer Registry (MCR) from 1990 -2014. Life tables from Statistics Canada were used as a reference for expected survival in the general population. Patient demographics were stratified by age, time period and residence at diagnosis. Two-year age-standardized relative survival estimates of patients in Manitoba with AML were estimated using the cohort method within various subcategories, including time period of diagnosis (in 5 year increments), sex and urban (Winnipeg) versus non-Winnipeg location. Results: A total of 910 patients from the ages of 15 to 99 were diagnosed from 1990 -2014 with available survival data to 2016. Of the 910 patients, 517 were men and 393 were women and 511 (56%) were residents of Winnipeg at diagnosis. Two-year crude relative survival estimates by time period and age group demonstrated improving relative survival for each age quintile category within each 5-year time period, with the most notable increase over time in patients aged 15-44 and 45-54. Total two-year age-standardized relative survival for all patients was 20.37% (CI 95% 17.81-23.05%). Two-year age-standardized relative survival in males was 19.22% (CI 95% 15.95-22.73%) and 21.93% (CI 95% 17.88-26.25%) in females. Two-year age-standardized relative survival by residence at diagnosis was 20.32% (CI 95% .16.92-23.94%) for urban (Winnipeg) residents, and 20.54% (CI 95% 16.74-24.62%) for those outside of Winnipeg. Summary/Conclusion: We demonstrate improved relative survival in adult AML over time, most notably in adults aged 15-54, where BMT has played an increasingly important therapeutic role. Advanced supportive care and hypomethylating agents may also have contributed to improved outcomes, especially in older patients. Similar relative survival for urban and rural patients supports the benefits of a centralized and coordinated leukemia centre with its closely linked community cancer program.
Background Haematological abnormalities are common in children with trisomy 21. These children have a remarkably high risk of acute leukaemia. The incidence of acute myeloid leukaemia is 150 fold greater in young children with DS compared to children of the same age without DS. Acute Megakaryoblastic Leukaemia (AMKL) is a subtype of myeloid leukaemia and is the most common leukaemia type in children with DS under 4 years of age. AMKL is often preceded by a transient neonatal pre-leukaemic syndrome, called Transient Myeloproliferative Disorder (TMD). Although TMD often spontaneously resolves, 20-30% of these patients subsequently develop AMKL within the first 4 years of life. Aims To perform a retrospective consecutive national audit of all documented cases of childhood TMD and AMKL-DS from 1990-2018 at the National Paediatric Haematology/Oncology Centre, Our Lady's Children's Hospital Crumlin (OLCHC) Ireland. We also aimed to compare our demographic and outcome findings with the recent medical literature and make recommendations for future research. Methods Charts of the patients with a diagnosis of AMKL and treated consecutively at (OLCHC) between 1990 -2018 were sourced from medical records. Charts and the hospital database were reviewed and information including date and age of diagnosis of AML, comorbidities, previous history of TMD, treatment regime and outcome was noted. The Hospital Patient Administration System (PAS) was interrogated for additional information such as percentage blasts in the bone marrow and blood count values at time of diagnosis of AMKL. Cytogenetic information was obtained by accessing a dedicated database. Kaplan-Meier survival curves were constructed. Results Twenty-seven patients with AMKL-DS were treated in OLCHC. A prior neonatal diagnosis of TMD was described in 10 patients (37%). Patients had a low platelet count (median 34 × 10 9 /L) at presentation. Nineteen patients (70%) are alive and well, in complete remission, at a median follow up of 11.4 years. Overall survival (OS) of this cohort has risen from 54% from those treated between the years 1990 -2004 (n = 13) to 93% for those treated between the years 2005 -2018 (n = 14). Conclusion High cure rates are observed in AMKL-DS using current polychemotherapy protocols. However, in general children with DS are more prone to the cytotoxic effects of chemotherapy, which can lead to significant treatment related mortality. The finding of a low platelet count at time of diagnosis is in keeping with the knowledge that AMKL-DS is a malignancy of platelet progenitor cells.
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