RESUMEN
Down syndrome (DS) is the commonest chromosomal disorder and is considered to be the most common syndrome associated with acute leukaemia. The objective of this study was to determine the characteristics of acute leukaemia in children with DS in Pakistan. It was a retrospective, cohort study conducted over a 2-year period, and the data was analysed in SPSS 20.0 in terms of descriptive statistics. Nineteen DS patients with acute leukaemia were enrolled. The proportion of DS-acute leukaemia was found to be 1.84% among all cases of paediatric acute leukaemia. The mean age of presentation was 5.5 years ± 4.3 SD with a male to female ratio of 1.1:1. The precursor B-cell ALL was found in 13 (68.4%) and acute myeloid leukaemia was found in 6 (31.6%) patients of DS. Thirteen patients (68.4%) completed treatment, while 6 (31.6%) expired due to treatment-related toxicity. Mean overall survival was 38 months ± 5.34 SD. The status of diagnosis of DS before presentation with acute leukaemia was the only statistically significant factor associated with the outcome. Few distinct characteristics of DS-acute leukaemia have been found in our population. Treatment toxicity was the sole cause of treatment failure.
RESUMEN
OBJECTIVE: To determine the proportion of treatment-related mortality among mortalities of paediatric acute lymphoblastic leukaemia and to identify probable causes and risk factors. METHODS: The observational retrospective study was conducted in February-March 2019 at the Department of Paediatric Haematology-Oncology and Bone Marrow Transplant, the Children's Hospital and the Institute of Child Health, Lahore, Pakistan, and comprised data of all paediatric patients of acute lymphoblastic leukaemia who expired during treatment from January 2017 till September 2018. Death due to relapse and deaths before treatment were excluded. Data was analysed using SPSS 16. RESULTS: Of the 247 deaths during the study period, 144(58.3%) were treatment-related mortality cases; 81(56.2%) males and 63(43.8%) females with an overall mean age of 5.0±3.83 years. The commonest cause was sepsis 126(87.5%), followed by haemorrhagic complications 11(7.6%), drug toxicity 4(2.8%), tumour lysis syndrome 2(1.4%) and thromboembolism 1(0.7%). Significant factors associated with treatment-related mortality were weight-for-age, immunophenotype, the reason for admission, and absolute neutrophil count (p<0.05). CONCLUSIONS: Treatment-related mortality, though potentially avoidable, was found to be a major cause of death among paediatric patients of acute lymphoblastic leukaemia, and sepsis was the most common cause.
