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t(3;11)(q28;q23)

Clinics and Pathology

Disease treatment related acute non lymphocytic leukemia (t-ANLL) so far
Epidemiology only 1 case available
Clinics a 41 yr old female patient with a previous history of non Hodgkin lymphoma treated withtopoisomerase II inhibitors 3 yrs earlier; she developped a M5a t-ANLL and died 10 days after diagnosis

Cytogenetics

Additional anomalies a complex karyotype was found in the only available case, with +8 and other anomalies.

Genes involved and Proteins

Gene Name LPP
Location 3q28
Protein LPP is a member of the LIM protein gene family, containing a leucin-zipper motif in its amino-terminal region and three LIM domains in its carboxy-terminal region
Gene Name MLL
Location 11q23
Protein contains two DNA binding motifs (a AT hook, and Zinc fingers), a DNA methyl transferase motif, a bromodomain; transcriptional regulatory factor involved in maintenance of Hox gene expression

Result of the chromosomal anomaly

Hybrid gene
Description 5' MLL including the 8 first exons fused to the 3 last 3' exons (exons 9 to 11) of LPP.
Transcript the reciprocal 5'MLPP-3'MLL is also transcribed
  
Fusion Protein
Description N-term AT hook and DNA methyltransferase from MLL fused to the 2 last C-term LIM domains of LPP
  

External links

Other databaset(3;11)(q28;q23) Mitelman database (CGAP - NCBI)
Other databaset(3;11)(q28;q23) CancerChromosomes (NCBI)

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

Human LPP gene is fused to MLL in a secondary acute leukemia with a t(3;11) (q28;q23).
Dahˆ©ron L, Veinstein A, Brizard F, Drabkin H, Lacotte L, Guilhot F, Larsen CJ, Brizard A, Roche J
Genes, chromosomes & cancer. 2001 ; 31 (4) : 382-389.
PMID 11433529
 

Contributor(s)

Written10-2001Jean-Loup Huret

Citation

This paper should be referenced as such :
Huret JL . t(3;11)(q28;q23). Atlas Genet Cytogenet Oncol Haematol. October 2001 .
URL : http://AtlasGeneticsOncology.org/Anomalies/t0311q28q23ID1223.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Mon May 12 18:12:13 2008


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