Atlas of Genetics and Cytogenetics in Oncology and Haematology


Home   Genes   Leukemias   Solid Tumors   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA

t(7;14)(q34;q11) TRA/TRB

Written2017-11Adriana Zamecnikova
Kuwait Cancer Control Center, Kuwait annaadria@yahoo.com

Abstract Translocations and inversions involving the TRA/TRD locus at 14q11.2 and the TRB locus at 7q34 are characteristic anomalies in T-cell acute leukemia/lymphoma. In most instances, juxtaposing promoter and enhancer elements of TCR genes in the proximity of a partner gene results in their dysregulation that may be a critical step toward development of T-cell malignancies.

Keywords T-cell lymphoblastic leukemia/lymphoma, t(7;14)(q34-35;q11), T- cell receptor genes.

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424
ICD-Morpho 9837/3 T lymphoblastic leukaemia/lymphoma
ICD-Morpho 9705/3 Angioimmunoblastic T-cell lymphoma
Atlas_Id 3284
 
  Figure 1. Partial karyotype with t(7;14)(q34;q11).

Clinics and Pathology

Note As translocation breakpoints near terminal regions of chromosomes are difficult to ascertain, included are cases with 7q35 breakpoints, probably involving TRB.
Disease T-cell acute lymphoblastic leukemia (T-ALL) and angioimmunoblastic T-cell lymphoma (AITL).
Etiology T-cell malignancies: 4 patients with T-cell acute lymphoblastic leukemia (Kaneko et al., 1988; Secker-Walker et al., 1992; Cauwelier et al., 2006) and 3 with angioimmunoblastic T-cell lymphoma (AITL) (Cosimi et al., 1990) (Table 1.).
Table 1. Clinical and genetic testing findings in reported patients with t(7;14)(q34-36;q11).
 Sex/AgeDiagnosisKaryotype/td>
1M/14T-ALL46,XY,t(7;14)(q35;q11) 
2F/18T-ALL46,XX,t(7;14)(q34;q11)
330/MT-ALL46-47,XY,+der(14)t(7;14)(q34;q11),+mar1   TRA-TRD/TRB
4F/5T-ALL45,X,?der(X),t(7;14)(q34;q11),-9,-9,del(11)(p11),del(12)(p?13),+mar   TRA-TRD/TRB
5FAITL46,XX,dup(7)(q11q35),t(7;14)(q35;q11),t(7;7)(p13;q35)   LN
6FAITL46,XX,dup(7)(q11q35),t(7;14)(q35;q11),t(7;14)(p13;q11),add(14)(q11)   LN
7FAITL48,XX,+3,+7,t(7;14)(q35;q11),t(7;14)(p13;q11),t(14;14)(q11;q32)   LN

1. Kaneko et al., 1988; 2. Secker-Walker et al., 1992; 3-4. Cauwelier et al., 2006; 5-7. Cosimi et al., 1990.
Abbreviations: M, male; F, female; AITL, Angioimmunoblastic T-cell lymphoma; LN, lymph node; T-ALL, T-cell Acute lymphoblastic leukemia.
Epidemiology 2 female and 1 male patients aged 5, 18 and 30 years old (Secker-Walker et al., 1992; Cauwelier et al., 2006). In addition 1 male (Kaneko et al., 1988) and 3 female patients (Cosimi et al., 1990) with 7q35 breakpoints were reported.
Prognosis Unknown due to the rarity of reported patients.

Cytogenetics

Cytogenetics Morphological Because in translocations with breakpoints in near terminal regions of chromosomes precise breakpoints are often difficult to define, cytogenetic appearance of t(7;14)(q34;q11) may resemble t(7;14)(q35;q11) and rearrangements of TRB at 7q34 may be detected only by using FISH with appropriate probes.
Sole anomaly in 2 (Kaneko et al., 1988; Secker-Walker et al 1992), complex karyotype in 1 (Cauwelier et al., 2006) and found as +der(14)t(7;14)(q34;q11),+mar1 in 1 T-ALL patient (Cauwelier et al., 2006). Found in association with complex anomalies including dup(7)(q11q35),t(7;7)(p13;q35),t(7;14)(p13;q11) and t(14;14)(q11;q32) in AITL patients (Cosimi et al., 1990).

Genes involved and Proteins

Gene NameTRB (T-cell receptor beta locus)
Location 7q34
Note Various genes are activated in T-cell malignancies as a consequence of chromosome translocations, inversions and cryptic chromosomal rearrangements involving sequences from the TRB locus such as: t(6;7)(q27;q34) TRB/ MYB, inv(7)(p15q34)/t(7;7)(p15;q34) TRB/ HOXA10, t(7;9)(q34;q34) TRB/ NOTCH1, t(7;10)(q34;q24) TRB/ HOXA11, t(7;11)(q34;p15) TRB/ LMO1, t(7;11)(q34;p13) TRB/ LMO2, t(7;14)(q34;q32.1) TRB/ TCL1A.
Gene NameTRA- TRD
Location 14q11.2
Note TRA/TRD is a target of chromosomal translocations and inversions including: inv(14)(q11q32.1)/t(14;14)(q11;q32.1) TRA-TRD/TCL1A, t(8;14)(q24.1;q11.2) MYC/TRA-TRD, t(10;14)(q24;q11.2) TLX1/TRA-TRD, t(11;14)(p15;q11.2) LMO1/TRA-TRD and t(11;14)(p13;q11.2) LMO2/TRA-TRD.

Result of the chromosomal anomaly

Fusion Protein
Oncogenesis Translocations between T-cell receptor genes and various oncogenes are a genetic hallmarks of T-cell malignancies. Chromosomal aberrations affecting TCR gene loci mainly involve TRA/TRD at 14q11.2 and TRB at 7q34 and affect various known oncogenes that most often code for transcription factors or proteins involved in transcriptional complexes, the deregulated expression of which is putatively implicated in T-cell oncogenesis.
  

To be noted

Additional cases are needed to delineate the epidemiology of this rare entity:
you are welcome to submit a paper to our new Case Report section.

Bibliography

Molecular cytogenetic study of 126 unselected T-ALL cases reveals high incidence of TCRbeta locus rearrangements and putative new T-cell oncogenes
Cauwelier B, Dastugue N, Cools J, Poppe B, Herens C, De Paepe A, Hagemeijer A, Speleman F
Leukemia 2006 Jul;20(7):1238-44
PMID 16673021
 
Rearrangements on chromosomes 7 and 14 with breakpoints at 7q35 and 14q11 in angioimmunoblastic lymphadenopathy and IBL-like T-cell lymphoma
Cosimi MF, Casagranda I, Ghiazza G, Rossi G, Galvani P
Pathologica 1990 Jul-Aug;82(1080):391-7
PMID 2284141
 
Chromosome translocations involving band 7q35 or 7p15 in childhood T-cell leukemia/lymphoma
Kaneko Y, Maseki N, Homma C, Sakurai M, Mizutani S, Takeda T, Shikano T, Fujimoto T, Yaoi K, Shimokawa T
Blood 1988 Aug;72(2):534-8
PMID 2900030
 
Karyotype and T-cell receptor expression in T-lineage acute lymphoblastic leukemia
Secker-Walker LM, Campana D, Hawkins JM, Sampson RE, Coustan-Smith E
Genes Chromosomes Cancer 1992 Jan;4(1):41-5
PMID 1377008
 

Citation

This paper should be referenced as such :
Zamecnikova A
t(7;14)(q34;q11) TRA/TRB;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0714q34q11ID3284.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(7;14)(q34;q11) TRA/TRB

External links

Mitelman databaset(7;14)(q34;q11) [Case List]    t(7;14)(q34;q11) [Transloc-MCList] TRA/TRB [Fusion-MCList]
arrayMap (UZH-SIB Zurich)Morph ( 9837/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMap (UZH-SIB Zurich)Morph ( 9705/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Fri Feb 23 20:21:57 CET 2018


Home   Genes   Leukemias   Solid Tumors   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.