Absence of Biallelic TCRgamma Deletion

MedGen UID:: 817139
•Concept ID:: C3811055
•: Cell or Molecular Dysfunction

Synonyms:

ABD; Absence of Biallelic TCRgamma Locus Deletion

Definition

The absence of rearrangement of at least one allele of the T-cell receptor gamma gene locus. This lack of rearrangement is characteristic of early thymocyte precursors and a lack of V(D)J recombination. This genetic profile is a predictor of induction therapy failure in children with T-cell acute lymphoblastic leukemia. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVAbsence of Biallelic TCRgamma Deletion

Molecular Abnormality
- Cytogenetic Abnormality
  - Chromosomal Rearrangement
    - Chromosome 7 Rearrangement
      - Clonal T-Cell Receptor Gamma Chain Gene Rearrangement
        Absence of Biallelic TCRgamma Deletion

Professional guidelines

PubMed

Absence of biallelic TCRgamma deletion predicts early treatment failure in pediatric T-cell acute lymphoblastic leukemia.

Gutierrez A, Dahlberg SE, Neuberg DS, Zhang J, Grebliunaite R, Sanda T, Protopopov A, Tosello V, Kutok J, Larson RS, Borowitz MJ, Loh ML, Ferrando AA, Winter SS, Mullighan CG, Silverman LB, Chin L, Hunger SP, Sallan SE, Look AT
J Clin Oncol 2010 Aug 20;28(24):3816-23. Epub 2010 Jul 19 doi: 10.1200/JCO.2010.28.3390. PMID: 20644084 Free PMC Article

See all (1)

Recent clinical studies

Etiology

Clinical impact of NOTCH1 and/or FBXW7 mutations, FLASH deletion, and TCR status in pediatric T-cell lymphoblastic lymphoma.

Callens C, Baleydier F, Lengline E, Ben Abdelali R, Petit A, Villarese P, Cieslak A, Minard-Colin V, Rullier A, Moreau A, Baruchel A, Schmitt C, Asnafi V, Bertrand Y, Macintyre E
J Clin Oncol 2012 Jun 1;30(16):1966-73. Epub 2012 Apr 30 doi: 10.1200/JCO.2011.39.7661. PMID: 22547598

Absence of biallelic TCRgamma deletion predicts early treatment failure in pediatric T-cell acute lymphoblastic leukemia.

See all (2)

Diagnosis

Clinical impact of NOTCH1 and/or FBXW7 mutations, FLASH deletion, and TCR status in pediatric T-cell lymphoblastic lymphoma.

Absence of biallelic TCRgamma deletion predicts early treatment failure in pediatric T-cell acute lymphoblastic leukemia.

See all (2)

Therapy

Clinical impact of NOTCH1 and/or FBXW7 mutations, FLASH deletion, and TCR status in pediatric T-cell lymphoblastic lymphoma.

Absence of biallelic TCRγ deletion predicts induction failure and poorer outcomes in childhood T-cell acute lymphoblastic leukemia.

Yang YL, Hsiao CC, Chen HY, Lin KH, Jou ST, Chen JS, Chang TK, Sheen JM, Yu SL, Lu MY, Cheng CN, Wu KH, Wang SC, Wang JD, Chang HH, Lin SR, Lin SW, Lin DT
Pediatr Blood Cancer 2012 Jun;58(6):846-51. Epub 2011 Dec 16 doi: 10.1002/pbc.24021. PMID: 22180181

Absence of biallelic TCRgamma deletion predicts early treatment failure in pediatric T-cell acute lymphoblastic leukemia.

See all (3)