Chapter title |
ETV6-RUNX1 + Acute Lymphoblastic Leukaemia in Identical Twins
|
---|---|
Chapter number | 14 |
Book title |
RUNX Proteins in Development and Cancer
|
Published in |
Advances in experimental medicine and biology, March 2017
|
DOI | 10.1007/978-981-10-3233-2_14 |
Pubmed ID | |
Book ISBNs |
978-9-81-103231-8, 978-9-81-103233-2
|
Authors |
Anthony M. Ford Ph.D., Mel Greaves FRS, Anthony M. Ford, Mel Greaves |
Editors |
Yoram Groner, Yoshiaki Ito, Paul Liu, James C. Neil, Nancy A. Speck, Andre van Wijnen |
Abstract |
Acute leukaemia is the major subtype of paediatric cancer with a cumulative risk of 1 in 2000 for children up to the age of 15 years. Childhood acute lymphoblastic leukaemia (ALL) is a biologically and clinically diverse disease with distinctive subtypes; multiple chromosomal translocations exist within the subtypes and each carries its own prognostic relevance. The most common chromosome translocation observed is the t(12;21) that results in an in-frame fusion between the first five exons of ETV6 (TEL) and almost the entire coding region of RUNX1 (AML1).The natural history of childhood ALL is almost entirely clinically silent and is well advanced at the point of diagnosis. It has, however, been possible to backtrack this process through molecular analysis of appropriate clinical samples: (i) leukaemic clones in monozygotic twins that are either concordant or discordant for ALL; (ii) archived neonatal blood spots or Guthrie cards from individuals who later developed leukaemia; and (iii) stored, viable cord blood cells.Here, we outline our studies on the aetiology and pathology of childhood ALL that provide molecular evidence for a monoclonal, prenatal origin of ETV6-RUNX1+ leukaemia in monozygotic identical twins. We provide mechanistic support for the concept that altered patterns of infection during early childhood can deliver the necessary promotional drive for the progression of ETV6-RUNX1+ pre-leukaemic cells into a postnatal overt leukaemia. |
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Unknown | 1 | 100% |
Demographic breakdown
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Unknown | 26 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Ph. D. Student | 4 | 15% |
Student > Master | 3 | 12% |
Student > Bachelor | 2 | 8% |
Researcher | 2 | 8% |
Student > Doctoral Student | 1 | 4% |
Other | 4 | 15% |
Unknown | 10 | 38% |
Readers by discipline | Count | As % |
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Biochemistry, Genetics and Molecular Biology | 5 | 19% |
Medicine and Dentistry | 5 | 19% |
Business, Management and Accounting | 2 | 8% |
Nursing and Health Professions | 1 | 4% |
Unspecified | 1 | 4% |
Other | 2 | 8% |
Unknown | 10 | 38% |