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t(3;11)(q28;q23) KMT2A/LPP

Written2001-10Jean-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 9807/3 Mixed phenotype acute leukaemia with t(v;11q23); MLL rearranged
ICD-Morpho 9813/3 B lymphoblastic leukaemia/lymphoma with t(v;11q23); MLL rearranged
ICD-Morpho 9920/3 Therapy-related myeloid neoplasms
Atlas_Id 1223

Clinics and Pathology

Disease treatment related acute myeloid leukemia (t-AML) 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-AML 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 KMT2A
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
  

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).
Dahéron 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
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;11)(q28;q23)
Atlas Genet Cytogenet Oncol Haematol. 2002;6(1):39-39.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0311q28q23ID1223.html


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

Genes LPP KMT2A

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;11)(q28;q23) KMT2A/LPP

External links

KMT2A (11q23.3) LPP (3q28)

Mitelman databaset(3;11)(q28;q23) [Case List]    t(3;11)(q28;q23) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9807/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9813/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9920/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
TICdbKMT2A/LPP  KMT2A (11q23.3) LPP (3q28)
 
Disease databaset(3;11)(q28;q23) KMT2A/LPP
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Tue Mar 14 13:54:39 CET 2017


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