t(10;11)(p12;q23) KMT2A/MLLT10

1997-12-01   Jean-Loup Huret  , Jean-Loup Huret  

1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology

Disease

AML

Phenotype stem cell origin

M5/M4; ALL at times, therapy related cases

Epidemiology

from infants to adults

Prognosis

poor

Cytogenetics

Cytogenetics morphological

A high diversity of reported breakpoints onchromosome 10, from 10p11 to 10p15: remains to be fully studiedL

Additional anomalies

inv(11)(q13q23) is frequently acompanying thet(10;11); this is likely to be related to the transcription directions ofthe two genes

Genes Involved and Proteins

Gene name
MLLT10 (myeloid/lymphoid or mixed-lineage leukemia (trithorax homolog, Drosophila); translocated to, 10)
Location
10p12.31
Dna rna description
5 telomeric --> 3 centromeric orientation
Protein description
contains 3 Zn fingezrs and a leucine zipper; nuclearlocalisation; transcription factor
Gene name
KMT2A (myeloid/lymphoid or mixed lineage leukemia)
Location
11q23.3
Dna rna description
21 exons, spanning over 100 kb; 13-15 kb mRNA
Protein description
431 kDa; contains two DNA binding motifs (a AT hook,
Protein description
and Zinc fingers), a DNA methyl transferase motif, a bromodomain;
Protein description
transcriptional regulatory factor; nuclear localisation

Result of the Chromosomal Anomaly

Description

5 MLL - 3 AF10; variable breakpoints on both chromosomes; thefusion transcript on chromosome 11 is the crucial one; MLL and AF10 are inopposite transcriptional directions, wich may be cause of the complexity ofthe translocationN-term AT hook and DNA methyltransferase from MLL fused to theleucine zipper C-term of AF10

Expression localisation

nuclear localisation

Highly cited references

Pubmed IDYearTitleCitations
302035712018KMT2A (MLL) rearrangements observed in pediatric/young adult T-lymphoblastic leukemia/lymphoma: A 10-year review from a single cytogenetic laboratory.12
302771152019Panoramic view of common fusion genes in a large cohort of Chinese de novo acute myeloid leukemia patients.9
307074742019Acute leukemias harboring KMT2A/MLLT10 fusion: a 10-year experience from a single genomics laboratory.8
345727622021Advances in the First Line Treatment of Pediatric Acute Myeloid Leukemia in the Polish Pediatric Leukemia and Lymphoma Study Group from 1983 to 2019.5
302737802019Accurate and Sensitive Analysis of Minimal Residual Disease in Acute Myeloid Leukemia Using Deep Sequencing of Single Nucleotide Variations.5
306248592019Acute myeloid leukemia with t(10;11)(p11-12;q23.3): Results of Russian Pediatric AML registration study.2
359785542022A rare case of transformation from relapsed acute monocytic leukaemia with KMT2A::MLLT10 to acute megakaryoblastic leukaemia.0
345514112022Adult Acute Myeloid Leukemia with the KMT2A-Mixed Lineage Leukemia T10 Fusion: An Analysis of 10 Cases Showed Common Features and Frequent Mutations in the RAS Signaling Pathway.0
289218162018Comprehensive genetic analysis of donor cell derived leukemia with KMT2A rearrangement.0
294455602017The Partial Duplication of the 5' Segment of KMT2A Revealed KMT2A-MLLT10 Rearrangement in a Boy with Acute Myeloid Leukemia.0

Bibliography

No bibliography items were found for this article.

Summary

Fusion gene

KMT2A/MLLT10 KMT2A (11q23.3) MLLT10 (10p12.31) COF 2033 2034 2035 2036 2037 2038 2039 2040 2041 2042 2043 2044 2045 2046 2047 2048 2049 2050 2051 2052|KMT2A/MLLT10 KMT2A (11q23.3) MLLT10 (10p12.31) TIC

Citation

Jean-Loup Huret ; Jean-Loup Huret

t(10;11)(p12;q23) KMT2A/MLLT10

Atlas Genet Cytogenet Oncol Haematol. 1997-12-01

Online version: http://atlasgeneticsoncology.org/haematological/1025/css/js/js/template.js