EQUAL FREQUENCY OF TEL/AML1 ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDREN WITH AND WITHOUT DOWN SYNDROME |
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| Authors: | Manuel Steiner Andishe Attarbaschi Margit K nig Helmut Gadner Oskar A. Haas Georg Mann |
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| Affiliation: | a St. Anna Children's Hospital, Vienna, Austriab Children's Cancer Research Institute, St. Anna Children's Hospital, Vienna, Austriac St. Anna Children's Hospital, Vienna, Austria; and Children's Cancer Research Institute, St. Anna Children's Hospital, Vienna, Austria |
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| Abstract: | Constitutional trisomy 21 is the most prominent predisposing factor to childhood leukemia, whereas the t(12;21)(p13;q22) with its molecular genetic counterpart, the TEL/AML1 fusion gene, is the most common acquired chromosomal rearrangement in childhood B-cell precursor (BCP) acute lymphoblastic leukemia (ALL). Thus, it was somewhat surprising that according to the currently available literature the incidence of TEL/AML1+ BCP ALL is extremely low in patients with Down syndrome (DS). To further investigate this issue in a population-based fashion, the authors retrospectively assessed the number of DS patients with a TEL/AML1+ ALL in two consecutive Austrian ALL multicenter trials. Accordingly, they were able to analyze 8 of 10 individuals with DS and a BCP ALL, including 2 who suffered from a TEL/AML1+ leukemia. Based on this observation we concluded that individuals with a constitutional trisomy 21 may have the similar likelihood to develop a TEL/AML1+ leukemia as BCP ALL patients without this specific predisposing factor. |
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| Keywords: | ALL Down syndrome incidence TEL/AML1 |
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