Minimal residual disease, long-term outcome, and IKZF1 deletions in children and adolescents with Down syndrome and acute lymphocytic leukaemia: a matched cohort study

Patients with Down syndrome and acute lymphocytic leukaemia are at an increased risk of treatment-related mortality and relapse, which is influenced by unfavourable genetic aberrations (eg, IKZF1 deletion). We aimed to investigate the potential underlying effect of Down syndrome versus the effects o...

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Published in:	The Lancet. Haematology 2021-10, Vol.8 (10), p.e700-e710
Main Authors:	Michels, Naomi, Boer, Judith M, Enshaei, Amir, Sutton, Rosemary, Heyman, Mats, Ebert, Sabine, Fiocco, Marta, de Groot-Kruseman, Hester A, van der Velden, Vincent H J, Barbany, Gisela, Escherich, Gabriele, Vora, Ajay, Trahair, Toby, Dalla-Pozza, Luciano, Pieters, Rob, zur Stadt, Udo, Schmiegelow, Kjeld, Moorman, Anthony V, Zwaan, C Michel, den Boer, Monique L
Format:	Article
Language:	eng
Subjects:	Medicin och hälsovetenskap
Online Access:	Request full text
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Summary:	Patients with Down syndrome and acute lymphocytic leukaemia are at an increased risk of treatment-related mortality and relapse, which is influenced by unfavourable genetic aberrations (eg, IKZF1 deletion). We aimed to investigate the potential underlying effect of Down syndrome versus the effects of adverse cancer genetics on clinical outcome. Patients (aged 1–23 years) with Down syndrome and acute lymphocytic leukaemia and matched non-Down syndrome patients with acute lymphocytic leukaemia (matched controls) from eight trials (DCOG ALL10 and ALL11, ANZCHOG ALL8, AIEOP-BFM ALL2009, UKALL2003, NOPHO ALL2008, CoALL 07-03, and CoALL 08-09) done between 2002 and 2018 across various countries (the Netherlands, the UK, Australia, Denmark, Finland, Iceland, Norway, Sweden, and Germany) were included. Participants were matched (1:3) for clinical risk factors and genetics, including IKZF1 deletion. The primary endpoint was the comparison of MRD levels (absolute MRD levels were categorised into two groups, low [
ISSN:	2352-3026 2352-3026