Authors: J. Zuna Authors‘ workplace: II. dětská klinika 2. LF UK a FNM, Praha Published in: Čas. Lék. čes. 1999; : 131-137 Category:
TEL and AML1 genes occur in a markedly high number of different aberrations in haematological malignancies.Besides the AML1, TEL is often fused to genes, which encod thyrosin-kinases. AML1 gene is a part of CBFtranscription factor. AML1 can be altered in childhood acute lymphoblastic leukaemia (ALL) and also in a substantialnumber of acute myeloid leukaemias (most frequently as an AML1/ETO fusion). TEL/AML1 fusion gene (derivedfrom t(12;21)(p13;q22) translocation) became recently one of the most important genetic aberrations in childrenwith ALL. TEL/AML1 act presumably as dominant inhibitors of the second AML1 allele and thus they blocktranscription of genes dependent on CBF factor. Childhood ALL with TEL/AML1 hybrid gene is very frequent(approximately 22 % of overall childhood ALL in the Czech Republic) and patients with this fusion form relativelyhomogenous group. These children are diagnosed mostly in pre-school age as a B cell precursor leukaemias and theyhave very good treatment results.
Key words: TEL, AML1, TEL/AML1 fusion gene, childhood acute lymphoblastic leukaemia.
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