Crosslineage TCR delta rearrangements occur shortly after the DJ joinings of the IgH genes in childhood precursor B ALL and display age-specific characteristics.
Br J Haematol
; 99(1): 115-21, 1997 Oct.
Article
in En
| MEDLINE
| ID: mdl-9359511
ABSTRACT
In order to test the hypothesis that the most immature T-cell receptor (TCR) rearrangements occur after the DJ joining of the immunoglobulin heavy chain genes (IgH), we analysed the TCR Vdelta2-Ddelta3 rearrangements in precursor B-cell leukaemias (PBC ALL) from 25 children younger than 3 years at disease onset and found that most of the junctional regions had N nucleotides inserted. We then selected 14 of these PCB ALLs for DJH (DJ joining of the IgH) characterization. These joining regions showed homology-directed recombination and lack of N regions, indicating absence of terminal deoxynucleotidyl transferase (TdT) activity during their rearrangement. Most leukaemias with a DJH rearrangement without N region have no, or only one, nucleotide in the joining regions of their Vdelta2-Ddelta3 rearrangements. The N regions of the TCR delta rearrangements displayed 'age-specific' differences in children younger than 3 years of age the N regions were shorter than in those older than 3 years, and the rearrangements frequently contained complete segments. We conclude that the Vdelta2-Ddelta3 rearrangement in childhood PCB ALLs is an early event following DJH rearrangement and that it occurs shortly before or after the first hit, leading to malignant transformation.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma
/
Immunoglobulin Heavy Chains
/
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Limits:
Adolescent
/
Child
/
Child, preschool
/
Humans
/
Infant
Language:
En
Journal:
Br J Haematol
Year:
1997
Document type:
Article
Affiliation country:
Austria