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Haematopoietic development and leukaemia in Down syndrome

Children with constitutional trisomy 21 (cT21, Down Syndrome, DS) are at a higher risk for both myeloid and B-lymphoid leukaemias. The myeloid leukaemias are often preceded by a transient neonatal pre-leukaemic syndrome, Transient Abnormal Myelopoiesis (TAM). TAM is caused by cooperation between cT21 and acquired somatic N-terminal truncating mutations in the key haematopoietic transcription factor GATA1. These mutations, which are not leukaemogenic in the absence of cT21, are found in almost one-third of neonates with DS. Analysis of primary human fetal liver haematopoietic cells and of human embryonic stem cells demonstrates that cT21 itself substantially alters human fetal haematopoietic development. Consequently, many haematopoietic developmental defects are observed in neonates with DS even in the absence of TAM. Although studies in mouse models have suggested a pathogenic role of deregulated expression of several chromosome 21-encoded genes, their role in human leukaemogenesis remains unclear. As cT21 exists in all embryonic cells, the molecular basis of cT21-associated leukaemias probably reflects a complex interaction between deregulated gene expression in haematopoietic cells and the fetal haematopoietic microenvironment in DS.

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о с т рый л е й к о з Haematopoietic development and leukaemia in Down syndrome Irene Roberts1 and Shai Izraeli2,3 1Paediatrics and Molecular Haematology Unit, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK; 2Childhood Leukaemia Research Unit, Department of Paediatric Haemato-Oncology, Cancer Research Centre, Sheba Medical Centre, Ramat Gan, Israel; 3 Department of human molecular genetics and biochemistry, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel Children with constitutional trisomy 21 (cT21, Down Syndrome, DS) are at a higher risk for both myeloid and B-lymphoid leukaemias. <...> The myeloid leukaemias are often preceded by a transient neonatal pre-leukaemic syndrome, Transient Abnormal Myelopoiesis (TAM). <...> TAM is caused by cooperation between cT21 and acquired somatic N-terminal truncating mutations in the key haematopoietic transcription factor GATA1. <...> These mutations, which are not leukaemogenic in the absence of cT21, are found in almost one-third of neonates with DS. Analysis of primary human fetal liver haematopoietic cells and of human embryonic stem cells demonstrates that cT21 itself substantially alters human fetal haematopoietic development. <...> Consequently, many haematopoietic developmental defects are observed in neonates with DS even in the absence of TAM. <...> Although studies in mouse models have suggested a pathogenic role of deregulated expression of several chromosome 21-encoded genes, their role in human leukaemogenesis remains unclear. <...> As cT21 exists in all embryonic cells, the molecular basis of cT21-associated leukaemias probably reflects a complex interaction between deregulated gene expression in haematopoietic cells and the fetal haematopoietic microenvironment in DS. Keywords: trisomy 21, Down syndrome, acute lymphoblastic leukaemia, acute megakaryoblastic leukaemia, transient abnormal myelopoiesis, GATA1 C hildren with constitutional trisomy 21 (cT21, Down syndrome, DS) have a remarkably high risk of acute leukaemia [1]. <...> The incidence of acute myeloid leukaemia (known as ML-DS) is c. 150 times greater in young children with DS compared to children of the same age without DS while the incidence of acute B-cell lymphoblastic leukaemia (B-ALL) is c. 33 times higher <...>
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