t(10;11)(p12;q21) PICALM/MLLT10

1998-01-01   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

a mainly T-cell ALL; at times AML and/or AML with T-cellcommon progenitor may be involved; FAB: L1/L2

Phenotype stem cell origin

a myelomonocytic/T- cell

Epidemiology

< 1% of ALL; about 5 % of T-ALL; sex ratio: 9M/5F (from 14 cases herein reviewed)

Clinics

organomegaly; no CNS involvement; blood data: high WBC (range 20-170 X 109/l)

Prognosis

median survival: 22 mths in this review; range: 0-33+ mths, n=11(but we are to be cautious: cases ascertained by molecular studies areneeded before true prognostic ascertainment)

Cytogenetics

Cytogenetics molecular

investigations are required

Additional anomalies

most often (11/15 cases) present; del(5q), +8, and +19already recurrent

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
PICALM (clathrin assembly lymphoid myeloid leukemia gene)
Location
11q14.2
Protein description
role in the integration of different signals;

Result of the Chromosomal Anomaly

Transcript

both 5 CALM - 3 AF10 and 5 AF10 - 3 CALM are expressed

Description

1- a 1595 amino acids protein with N-term and most of CALM(except the last 4 amino acids!) fused to most of AF10 from amino acid 81(excluding the N-term zinc finger of AF10) C-term; 2- and a small 84 aminoacids protein with N-term zinc finger from AF10 fused to the very C-termend of CALM

Oncogenesis

it is not known which of the 2 fusion protein has the critical role

Bibliography

No bibliography items were found for this article.

Summary

Fusion gene

PICALM/MLLT10 PICALM (11q14.2) MLLT10 (10p12.31) M t(10;11)(p12;q14) t(10;11)(p12;q23) t(10;19;11)(p12;p13;q14) t(9;11)(p12;q14)|PICALM/MLLT10 PICALM (11q14.2) MLLT10 (10p12.31) TF LAML|PICALM/MLLT10 PICALM (11q14.2) MLLT10 (10p12.31) TIC

Note

the description opf this rare entity is arduous, since cases without molecular studies can be confused with cases of t(10;11)(p12;q23)
Atlas Image
t(10;11)(p13;q21) G-banding (top 2) - Courtesy Diane H. Norback, Eric B. Johnson, Sara Morrison-Delap UW Cytogenetic Services; middle row: - Courtesy Catherine Roche-Lestienne. R-banding (bottom 2) and Fish studies with MLL probe showing a signal on the normal 11 and one on the der(10) (right) and with chromosome 11 paint - Courtesy Pascale Cornillet-Lefebvre and Stéphanie Struski.

Citation

Jean-Loup Huret ; Jean-Loup Huret

t(10;11)(p12;q21) PICALM/MLLT10

Atlas Genet Cytogenet Oncol Haematol. 1998-01-01

Online version: http://atlasgeneticsoncology.org/haematological/1067/t(10;11)(p12;q21)-picalm-mllt10

External Links