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Shwachman–Diamond syndrome: implications for understanding the molecular basis of leukaemia

Published online by Cambridge University Press:  23 December 2008

Yigal Dror
Yigal Dror
Affiliation:
Cell Biology Program, Research Institute and the Marrow Failure and Myelodysplasia Program, Division of Haematology/Oncology, The Hospital for Sick Children and the University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. Tel. +1 416 813 8886; Fax: +1 416 813 5327; E-mail: yigal.dror@sickkids.ca
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Abstract

Inherited bone marrow failure syndromes provide extremely useful genetic models for understanding leukaemogenesis because the initial genetic defect can be identified and the risk of leukaemia is very high. Shwachman–Diamond syndrome is one of the most common inherited bone marrow failure syndromes and an example of such a model. Here, I describe the malignant features of Shwachman–Diamond syndrome and discuss the potential molecular mechanisms that can lead to leukaemia.

Type
Review Article
Information
Expert Reviews in Molecular Medicine , Volume 10 , October 2008 , e38
Copyright
Copyright © Cambridge University Press 2008

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References

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Further reading, resources and contacts

Bagby, G.C. and Meyers, G. (2007) Bone marrow failure as a risk factor for clonal evolution, prospects for leukemia prevention. Hematology American Society of Hematology Education Program Book 2007, 40-46.CrossRefGoogle Scholar
Bagby, G.C. and Meyers, G. (2007) Bone marrow failure as a risk factor for clonal evolution, prospects for leukemia prevention. Hematology American Society of Hematology Education Program Book 2007, 40-46.CrossRefGoogle Scholar