Download Leukaemia Section t(6;11)(q27;q23) Atlas of Genetics and Cytogenetics in Oncology and Haematology

Atlas of Genetics and Cytogenetics
in Oncology and Haematology
OPEN ACCESS JOURNAL AT INIST-CNRS
Leukaemia Section
Mini Review
t(6;11)(q27;q23)
Jean-Loup Huret
Genetics, Department of Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021
Poitiers, France
Published in Atlas Database: November 1997
Online version is available at: http://AtlasGeneticsOncology.org/Anomalies/t0611.html
DOI: 10.4267/2042/32066
This work is licensed under a Creative Commons Attribution-Non-commercial-No Derivative Works 2.0 France Licence.
© 1997 Atlas of Genetics and Cytogenetics in Oncology and Haematology
Identity
t(6;11)(q27;q23) G-banding - Courtesy Diane H. Norback, Eric B. Johnson, Sara Morrison-Delap Cytogenetics at the Waisman Center
(top and middle), and Jean-Luc Lai (below).
Epidemiology
Still poorly known; found in children and young adults;
male predominance.
Clinics
Frequent infections; organomegaly; blood data:
moderate WBC.
Clinics and pathology
Disease
ANLL
Phenotype / cell stem origin
M4, M5 mostly; T-cell ALL at times; therapy related
AL.
Atlas Genet Cytogenet Oncol Haematol. 1997;1(2)
87
t(6;11)(q27;q23)
Huret JL
hook and Zinc fingers), and a DNA methyl transferase
motif; wide expression; nuclear localisation;
transcriptional regulatory factor.
Treatment
BMT is indicated.
Prognosis
Very poor (as in other 11q23 rearrangements); rare
remission; short survival.
Results of the chromosomal
anomaly
Cytogenetics
Hybrid gene
Cytogenetics, morphological
Description
5’ MLL - 3’ AF6
May be missinterpreted as a del(11q), as chromosome 6
involvement may be overlooked.
Fusion protein
Cytogenetics, molecular
Description
About 1400 NH2-term amino acids (with the AT hook
and DNA binding motifs) from MLL and most of AF6,
starting at amino acid 26.
Therefore, FISH may be needed.
Additional anomalies
Are present in most cases; +8 in particular; +3; +19;
+21.
References
Genes involved and Proteins
Prasad R, Gu Y, Alder H, Nakamura T, Canaani O, Saito H,
Huebner K, Gale RP, Nowell PC, Kuriyama K, et al. Cloning of
the ALL-1 fusion partner, the AF-6 gene, involved inacute
myeloid leukemias with the t(6;11) chromosome translocation.
Cancer Res 1993 Dec 1;53(23):5624-8.
AF6
Location: 6q27
Protein
Contains a GLGF motif; widely expressed; cytoplasmic
localisation; role in signal transduction.
Welborn JL, Jenks HM, Hagemeijer A. Unique clinical features
and prognostic significance of the translocation (6;11) in acute
leukemia. Cancer Genet Cytogenet 1993 Feb;65(2):125-9.
(Review).
MLL
This article should be referenced as such:
Location: 11q23
Protein
431 kDa; contains two DNA binding motifs (a AT
Atlas Genet Cytogenet Oncol Haematol. 1997;1(2)
Huret JL. t(6;11)(q27;q23). Atlas Genet Cytogenet Oncol
Haematol.1997;1(2):87-88.
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