t(8;14)(q24;q11) TRA/MYC

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t(8;14)(q24;q11) TRA/MYC

Written	2001-04	Jacques Boyer
		Laboratoire d' hématologie, CH du MANS, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS

ICD-Morpho 9811/3 B lymphoblastic leukaemia/lymphoma, NOS

ICD-Morpho 9837/3 T lymphoblastic leukaemia/lymphoma

Atlas_Id 1061

t(8;14)(q24;q11) G-banding - courtesy Charles Bangs and Leena Borkar, and R-banding (right) - courtesy Jacques Boyer.

Clinics and Pathology

Disease acute lymphoblastic leukemia (ALL), (rare lymphoblastic lymphoma)

Phenotype / cell stem origin T lineage ( TCR alpha beta + or TCR gamma delta ). May be early-B lineage : conceivably the rare cases with early-B lineage represent a bipotential B-T lineage.

Epidemiology rare: prevalence 0.5 to 1.3 % among all cases off ALL and about 2% among T ALL; male predominance; median age 5 years

Clinics bulky extramedullary leukemia, central nervous system (CNS) infiltration

Cytology high white blood cell count

Prognosis the disease progress rapidly and response to conventional therapy is poor, median survival 11 months

Cytogenetics

Additional anomalies sole anomaly in half cases; additional anomalies are variable

Genes involved and Proteins

Note on the molecular point of view the proto oncogen c-myc is juxtaposed with the gene of the T-cell receptor alpha chain (TCR-alpha)

Gene Name MYC

Location 8q24

Dna / Rna c-myc has three exons, two promoters P1 and P2 control the c-myc transcription

Protein Myc protein is a transcription factor of the helix loop leucin zipper family that activates transcription as obligate heterodimer with a partner protein, Max

Gene Name TCR-alpha

Location 14q11.2

Dna / Rna the TCR delta variable (V) diversity (D) joining (J) and constant region genes are situated within the TCR alpha locus between the TCR alpha V and the TCR alpha J segments. The TCR alpha/delta locus is transcribed in a centromer to telomer direction.

Protein T-cell receptor

Gene Name TCR-delta

Location 14q11.2

Dna / Rna the TCR delta variable (V) diversity (D) joining (J) and constant region genes are situated within the TCR alpha locus between the TCR alpha V and the TCR alpha J segments. The TCR alpha/delta locus is transcribed in a centromer to telomer direction.

Protein T-cell receptor

Result of the chromosomal anomaly

Hybrid gene
Description the majority of breakpoints are localised within the TCR delta gene although they may also involve TCR alpha J and rarely TCR alpha V. The 3' part of the TCR alpha gene is relocated downstream of the c-myc protooncogene. The c-myc oncogene is not structurally altered.

Transcript detection of TCR alpha rearrangements is hampered by the large number of J segments

Fusion Protein
Note no fusion protein but promoter exchange

Description high levels of electrophoretically normal p64 and p67 c-myc proteins are detected and both products keep their instability. Preferential utilization of P2 is maintained.

Oncogenesis the activation of the gene myc is likely to result from its juxtaposition to the enhancer element of the TCR alpha gene with stimulates constitutive synthesis of normal c-myc.

To be noted

the identification of a breakpoint to the 3' side of c-myc suggest that this translocation is analogous to the t(2;8) and t(8;22) in Burkitt lymphoma. It is hypothesized that quantitative alteration of c-myc transcription alone may be sufficient for altered growth.

Bibliography

Cytogenetic abnormalities in adult acute lymphoblastic leukemia: correlations with hematologic findings outcome. A Collaborative Study of the Groupe Français de Cytogénétique Hématologique (GFCH).

Blood. 1996 ; 87 (8) : 3135-3142.

PMID 8605327

Molecular mechanisms of a t(8;14)(q24;q11) translocation juxtaposing c-myc and TcR-alpha genes in a T-cell leukaemia: involvement of a V alpha internal heptamer.

Bernard O, Larsen CJ, Hampe A, Mauchauffé M, Berger R, Mathieu-Mahul D

Oncogene. 1988 ; 2 (2) : 195-200.

PMID 2966921

A new case of acute lymphoblastic leukemia B-cell type with chromosomal rearrangements involving the T-cell receptor breakpoint at band 14q11.

Cigudosa JC, Calasanz MJ, Gullón A, Prósper F, Cuesta B, Rifón J, Rocha E

American journal of hematology. 1992 ; 41 (2) : 137-139.

PMID 1415175

Deregulation of c-myc by translocation of the alpha-locus of the T-cell receptor in T-cell leukemias.

Erikson J, Finger L, Sun L, ar-Rushdi A, Nishikura K, Minowada J, Finan J, Emanuel BS, Nowell PC, Croce CM

Science (New York, N.Y.). 1986 ; 232 (4752) : 884-886.

PMID 3486470

Translocation between chromosomes 8q24 and 14q11 in T-cell acute lymphoblastic leukemia.

Inaba T, Murakami S, Oku N, Itoh K, Ura Y, Nakanishi S, Shimazaki C, Nishio A, Nakagawa M, Fujita N

Cancer genetics and cytogenetics. 1990 ; 49 (1) : 69-74.

PMID 2397475

Rearrangements in the human T-cell-receptor alpha-chain locus in patients with adult T-cell leukemia carrying translocations involving chromosome 14q11.

Isobe M, Sadamori N, Russo G, Shimizu S, Yamamori S, Itoyama T, Yamada Y, Ikeda S, Ichimaru M, Kagan J

Cancer research. 1990 ; 50 (19) : 6171-6175.

PMID 2169336

Molecular involvement of the pvt-1 locus in a gamma/delta T-cell leukemia bearing a variant t(8;14)(q24;q11) translocation.

Kasai M, Maziarz RT, Aoki K, Macintyre E, Strominger JL

Molecular and cellular biology. 1992 ; 12 (10) : 4751-4757.

PMID 1406658

Pediatric leukemia/lymphoma with t(8;14)(q24;q11).

Lange BJ, Raimondi SC, Heerema N, Nowell PC, Minowada J, Steinherz PE, Arenson EB, O'Connor R, Santoli D

Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1992 ; 6 (7) : 613-618.

PMID 1385638

A t(8;14)(q24;q11) translocation in a T-cell leukemia (L1-ALL) with c-myc and TcR-alpha chain locus rearrangements.

Mathieu-Mahul D, Sigaux F, Zhu C, Bernheim A, Mauchauffe M, Daniel MT, Berger R, Larsen CJ

International journal of cancer. Journal international du cancer. 1986 ; 38 (6) : 835-840.

PMID 3466870

Molecular cloning of the breakpoint junction of a human chromosomal 8;14 translocation involving the T-cell receptor alpha-chain gene and sequences on the 3' side of MYC.

McKeithan TW, Shima EA, Le Beau MM, Minowada J, Rowley JD, Diaz MO

Proceedings of the National Academy of Sciences of the United States of America. 1986 ; 83 (17) : 6636-6640.

PMID 3529089

An (8;14)(q24;q11) translocation involving the T-cell receptor alpha-chain gene and the MYC oncogene 3' region in a B-cell lymphoma.

Park JK, McKeithan TW, Le Beau MM, Bitter MA, Franklin WA, Rowley JD, Diaz MO

Genes, chromosomes & cancer. 1989 ; 1 (1) : 15-22.

PMID 2535034

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 ; 4 (1) : 41-45.

PMID 1377008

Gene encoding the alpha chain of the T-cell receptor is moved immediately downstream of c-myc in a chromosomal 8;14 translocation in a cell line from a human T-cell leukemia.

Shima EA, Le Beau MM, McKeithan TW, Minowada J, Showe LC, Mak TW, Minden MD, Rowley JD, Diaz MO

Proceedings of the National Academy of Sciences of the United States of America. 1986 ; 83 (10) : 3439-3443.

PMID 3517860

Molecular analysis of the t(8;14)(q24;q11) chromosomal breakpoint junctions in the T-cell leukemia line MOLT-16.

Shima-Rich EA, Harden AM, McKeithan TW, Rowley JD, Diaz MO

Genes, chromosomes & cancer. 1997 ; 20 (4) : 363-371.

PMID 9408752

c-myc gene expression in a leukemic T-cell line bearing a t(8;14) (q24;q11) translocation.

Soudon J, Bernard O, Mathieu-Mahul D, Larsen CJ

Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1991 ; 5 (1) : 60-65.

PMID 1705637

Citation

This paper should be referenced as such :

Boyer, J

t(8;14)(q24;q11)

Atlas Genet Cytogenet Oncol Haematol. 2001;5(3):190-191.

Free journal version : [ pdf ] [ DOI ]

On line version : http://AtlasGeneticsOncology.org/Anomalies/t0814ID1061.html

Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes TRA TRD

Translocations implicated (Data extracted from papers in the Atlas)

t(8;14)(q24;q11) TRA/MYC

External links

TRA (-) MYC (8q24.21)
Mitelman database t(8;14)(q24;q11) [Case List] t(8;14)(q24;q11) [Association List] Mitelman database (CGAP - NCBI)
arrayMap Topo ( C42) Morph ( 9811/3) - arrayMap (UZH-SIB Zurich) [auto + random 100 samples .. if exist ] [tabulated segments]

arrayMap Topo ( C42) Morph ( 9837/3) - arrayMap (UZH-SIB Zurich) [auto + random 100 samples .. if exist ] [tabulated segments]

Mitelman database TRA/MYC [MCList] TRA (-) MYC (8q24.21)

Disease database t(8;14)(q24;q11) TRA/MYC

REVIEW articles automatic search in PubMed

Last year articles automatic search in PubMed

All articles automatic search in PubMed

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For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.

ICD-Topo	C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho	9811/3 B lymphoblastic leukaemia/lymphoma, NOS
ICD-Morpho	9837/3 T lymphoblastic leukaemia/lymphoma
Atlas_Id	1061


	t(8;14)(q24;q11) G-banding - courtesy Charles Bangs and Leena Borkar, and R-banding (right) - courtesy Jacques Boyer.

Disease	acute lymphoblastic leukemia (ALL), (rare lymphoblastic lymphoma)
Phenotype / cell stem origin	T lineage ( TCR alpha beta + or TCR gamma delta ). May be early-B lineage : conceivably the rare cases with early-B lineage represent a bipotential B-T lineage.
Epidemiology	rare: prevalence 0.5 to 1.3 % among all cases off ALL and about 2% among T ALL; male predominance; median age 5 years
Clinics	bulky extramedullary leukemia, central nervous system (CNS) infiltration
Cytology	high white blood cell count
Prognosis	the disease progress rapidly and response to conventional therapy is poor, median survival 11 months

Gene Name	MYC
Location	8q24
Dna / Rna	c-myc has three exons, two promoters P1 and P2 control the c-myc transcription
Protein	Myc protein is a transcription factor of the helix loop leucin zipper family that activates transcription as obligate heterodimer with a partner protein, Max

Gene Name	TCR-alpha
Location	14q11.2
Dna / Rna	the TCR delta variable (V) diversity (D) joining (J) and constant region genes are situated within the TCR alpha locus between the TCR alpha V and the TCR alpha J segments. The TCR alpha/delta locus is transcribed in a centromer to telomer direction.
Protein	T-cell receptor

Gene Name	TCR-delta
Location	14q11.2
Dna / Rna	the TCR delta variable (V) diversity (D) joining (J) and constant region genes are situated within the TCR alpha locus between the TCR alpha V and the TCR alpha J segments. The TCR alpha/delta locus is transcribed in a centromer to telomer direction.
Protein	T-cell receptor

Description	the majority of breakpoints are localised within the TCR delta gene although they may also involve TCR alpha J and rarely TCR alpha V. The 3' part of the TCR alpha gene is relocated downstream of the c-myc protooncogene. The c-myc oncogene is not structurally altered.
Transcript	detection of TCR alpha rearrangements is hampered by the large number of J segments

Note	no fusion protein but promoter exchange
Description	high levels of electrophoretically normal p64 and p67 c-myc proteins are detected and both products keep their instability. Preferential utilization of P2 is maintained.
Oncogenesis	the activation of the gene myc is likely to result from its juxtaposition to the enhancer element of the TCR alpha gene with stimulates constitutive synthesis of normal c-myc.

Mitelman database	t(8;14)(q24;q11) [Case List] t(8;14)(q24;q11) [Association List] Mitelman database (CGAP - NCBI)
arrayMap	Topo ( C42) Morph ( 9811/3) - arrayMap (UZH-SIB Zurich) [auto + random 100 samples .. if exist ] [tabulated segments]
arrayMap	Topo ( C42) Morph ( 9837/3) - arrayMap (UZH-SIB Zurich) [auto + random 100 samples .. if exist ] [tabulated segments]

Mitelman database	TRA/MYC [MCList] TRA (-) MYC (8q24.21)

Disease database	t(8;14)(q24;q11) TRA/MYC

REVIEW articles	automatic search in PubMed
Last year articles	automatic search in PubMed
All articles	automatic search in PubMed