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t(14;20)(q32;q13) IGH/CEBPB

Written2008-06Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, 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
Atlas_Id 1439

Clinics and Pathology

Phenotype / cell stem origin CD10+ acute lymphoblastic leukaemia (B-ALL)
Epidemiology Only four cases to date (Martineau et al., 1996; Akasaka et al., 2007), one male patient and 3 female patients, aged 11, 13, 15, and 35 yrs.
Clinics There was a high WBC in 2 of 3 cases.
Prognosis Survival was 7 mths+, 42 mths+, and 87 mths+.

Genetics

In the 3 cases reported by Akasaka et al (2007), a CEBPB / IGH involvement was uncovered.

Genes involved and Proteins

Gene NameCEBPB (CCAAT/enhancer binding protein beta)
Location 20q13.13
Protein DNA-binding protein. CCAAT enhancer-binding protein (CEBP) transcription factors are a family of 6 multifunctional basic leucine zipper (bZIP) transcription factors. The 5 other CEBPs are: CEBPA (19q13), CEBPD (8q11), CEBPE (8q11), CEBPG (19q13), all four equally implicated in leukemias, and DDIT3/CHOP/CEBP zeta (12q13), so far known to be involved in solid tumours (liposarcoma). These transcription factors play a key role in cellular differentiation, in particular in the control of myeloid differentiation. CEBPB is composed of a N-term transactivation domain, a negative regulatory domain, a DNA-binding basic motif, and a leucine-zipper domain in C-term. Different isoforms (LAP: liver activating protein, and LIP: liver inhibitory protein). CEBPB suppresses cell proliferation through repression of E2F target genes; on the other hand, CEBPB may promote tumorization of epithelial cells (Ramji et al., 2002; Nerlov et al., 2007).
Gene NameIGH (Immunoglobulin Heavy)
Location 14q32.33

Result of the chromosomal anomaly

Fusion Protein
Oncogenesis Overexpression of the CEBP gene.
  

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

Five members of the CEBP transcription factor family are targeted by recurrent IGH translocations in B-cell precursor acute lymphoblastic leukemia (BCP-ALL).
Akasaka T, Balasas T, Russell LJ, Sugimoto KJ, Majid A, Walewska R, Karran EL, Brown DG, Cain K, Harder L, Gesk S, Martin-Subero JI, Atherton MG, Bruggemann M, Calasanz MJ, Davies T, Haas OA, Hagemeijer A, Kempski H, Lessard M, Lillington DM, Moore S, Nguyen-Khac F, Radford-Weiss I, Schoch C, Struski S, Talley P, Welham MJ, Worley H, Strefford JC, Harrison CJ, Siebert R, Dyer MJ.
Blood. 2007 Apr 15; 109(8): 3451-61. Epub 2006 Dec 14.
PMID 17170124
 
Isochromosomes in acute lymphoblastic leukaemia: i(21q) is a significant finding.
Martineau M, Clark R, Farrell DM, Hawkins JM, Moorman AV, Secker-Walker LM.
Genes Chromosomes Cancer. 1996 Sep; 17(1): 21-30.
PMID 8889503
 
The C/EBP family of transcription factors: a paradigm for interaction between gene expression and proliferation control.
Nerlov C.
Trends Cell Biol. 2007 Jul; 17(7): 318-24. Epub 2007 Jul 19. (Review)
PMID 17658261
 
CCAAT/enhancer-binding proteins: structure, function and regulation.
Ramji DP, Foka P.
Biochem J. 2002 Aug 1; 365(Pt 3): 561-75. (Review)
PMID 12006103
 

Citation

This paper should be referenced as such :
Huret JL
t(14;20)(q32;q13) IGH/CEBPB;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t1420q32q13CEBPBIGHID1439.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(14;20)(q32;q13) IGH/CEBPB

External links

IGH (14q32.33) CEBPB (20q13.13)

Mitelman databaset(14;20)(q32;q13) [Case List]    t(14;20)(q32;q13) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9811/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
Mitelman databaseIGH/CEBPB [MCList]  IGH (14q32.33) CEBPB (20q13.13)
 
Disease databaset(14;20)(q32;q13) IGH/CEBPB
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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