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Br J Haematol. 2018 Nov;183(4):618-628. doi: 10.1111/bjh.15587. Epub 2018 Nov 8.

Complex and monosomal karyotype are distinct cytogenetic entities with an adverse prognostic impact in paediatric acute myeloid leukaemia. A NOPHO-DBH-AML study.

Author information

1
Department of Paediatrics and Adolescent Medicine, Aarhus University Hospital, Aarhus, Denmark.
2
Institution for Clinical Sciences, Department of Paediatrics, Queen Silvia Children's Hospital, Gothenburg, Sweden.
3
Department of Cytogenetics, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
4
Department of Paediatrics, University Medical Centre Groningen, Groningen, the Netherlands.
5
Hong Kong Paediatric Haematology & Oncology Study Group, Department of Paediatrics, Queen Mary Hospital, Hong Kong, China.
6
Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
7
Department of Paediatrics, Landspitalinn, Reykjavik, Iceland.
8
Paediatric Oncology/Haematology, VU University Medical Centre, Amsterdam, Academy of Princess Maxima Centre for Paediatric Oncology, Utrecht, The Netherlands.
9
Department of Paediatrics, Children's Clinical University Hospital, Riga, Latvia.
10
Department of Paediatrics and Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
11
Department of Paediatrics, Ghent University Hospital, Ghent, Belgium.
12
Department of Medical Biosciences, Genetics, Umeå University Hospital, Umeå, Sweden.
13
Department of Woman's and Children's Health, Uppsala University, Uppsala, Sweden.
14
Department of Paediatrics, SA Tallinna Lastehaigla, Tallinn, Estonia.
15
Division of Paediatric and Adolescent Medicine, Oslo University Hospital, Oslo, Norway.
16
Department of Cytogenetics, Aarhus University Hospital, Aarhus, Denmark.

Abstract

Data on occurrence, genetic characteristics and prognostic impact of complex and monosomal karyotype (CK/MK) in children with acute myeloid leukaemia (AML) are scarce. We studied CK and MK in a large unselected cohort of childhood AML patients diagnosed and treated according to Nordic Society for Paediatric Haematology and Oncology (NOPHO)-AML protocols 1993-2015. In total, 800 patients with de novo AML were included. CK was found in 122 (15%) and MK in 41 (5%) patients. CK and MK patients were young (median age 2·1 and 3·3 years, respectively) and frequently had FAB M7 morphology (24% and 22%, respectively). Refractory disease was more common in MK patients (15% vs. 4%) and stem cell transplantation in first complete remission was more frequent (32% vs. 19%) compared with non-CK/non-MK patients. CK showed no association with refractory disease but was an independent predictor of an inferior event-free survival (EFS; hazard ratio [HR] 1·43, P = 0·03) and overall survival (OS; HR 1·48, P = 0·01). MK was associated with a poor EFS (HR 1·57, P = 0·03) but did not show an inferior OS compared to non-MK patients (HR 1·14, P = 0·62). In a large paediatric cohort, we characterized AML with non-recurrent abnormal karyotype and unravelled the adverse impact of CK and MK on prognosis.

KEYWORDS:

Acute myeloid leukaemia; complex karyotype; monosomal karyotype; paediatrics; refractory disease

PMID:
30406946
DOI:
10.1111/bjh.15587
[Indexed for MEDLINE]

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