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t(10;11)(p13;q21) PICALM/MLLT10

Written1998-01Jean-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 9811/3 B lymphoblastic leukaemia/lymphoma, NOS
ICD-Morpho 9837/3 T lymphoblastic leukaemia/lymphoma
ICD-Morpho 9861/3 AML with mutated NPM1; AML with mutated CEBPA; Acute myeloid leukaemia, NOS
Atlas_Id 1067
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)
 
  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.

Clinics and Pathology

Disease a mainly T-cell ALL; at times AML and/or AML with T-cell markers, or B-cell ALL common progenitor may be involved; FAB: L1/L2
Phenotype / cell stem 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 are needed before true prognostic ascertainment)

Cytogenetics

Cytogenetics Molecular investigations are required
Additional anomalies most often (11/15 cases) present; del(5q), +8, and +19 already recurrent

Genes involved and Proteins

Gene NameMLLT10 (myeloid/lymphoid or mixed-lineage leukemia (trithorax homolog, Drosophila); translocated to, 10)
Location 10p12.31
Dna / Rna 5' telomeric --> 3' centromeric orientation
Protein contains 3 Zn fingezrs and a leucine zipper; nuclear localisation; transcription factor
Gene NamePICALM (clathrin assembly lymphoid myeloid leukemia gene)
Location 11q14.2
Protein role in the integration of different signals;

Result of the chromosomal anomaly

Hybrid gene
Transcript both 5' CALM - 3' AF10 and 5' AF10 - 3' CALM are expressed
  
Fusion Protein
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 amino acids protein with N-term zinc finger from AF10 fused to the very C-term end of CALM
Oncogenesis it is not known which of the 2 fusion protein has the critical role
  

Bibliography

t(10;11)(p13-14;q14-21): a new recurrent translocation in T-cell acute lymphoblastic leukemias. Groupe Français de Cytogénétique Hématologique (GFCH).
Genes, chromosomes & cancer. 1991 ; 3 (6) : 411-415.
PMID 1777411
 
Hematologic malignancies with the t(10;11) (p13;q21) have the same molecular event and a variety of morphologic or immunologic phenotypes.
Kobayashi H, Hosoda F, Maseki N, Sakurai M, Imashuku S, Ohki M, Kaneko Y
Genes, chromosomes & cancer. 1997 ; 20 (3) : 253-259.
PMID 9365832
 
Acute leukemia with t(10;11)(p11-p15;q13-q23).
Secco C, Wiernik PH, Bennett JM, Paietta E
Cancer genetics and cytogenetics. 1996 ; 86 (1) : 31-34.
PMID 8616782
 

Citation

This paper should be referenced as such :
Huret, JL
t(10;11)(p13;q21)
Atlas Genet Cytogenet Oncol Haematol. 1998;2(1):32-33.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t1011bisID1067.html


Other genes implicated (Data extracted from papers in the Atlas) [ 4 ]

Genes MLLT10 PICALM DOT1L PIMREG

Translocations implicated (Data extracted from papers in the Atlas)

 t(10;11)(p13;q21) PICALM/MLLT10

External links

PICALM (11q14.2) MLLT10 (10p12.31)

PICALM (11q14.2) MLLT10 (10p12.31)

PICALM (11q14.2) MLLT10 (10p12.31)

Mitelman databaset(10;11)(p13;q21) [Case List]    t(10;11)(p13;q21) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9811/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9837/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9861/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
Mitelman databasePICALM/MLLT10 [MCList]  PICALM (11q14.2) MLLT10 (10p12.31)
TCGA_FusionPICALM/MLLT10 [LAML]  PICALM (11q14.2) MLLT10 (10p12.31)
TICdbPICALM/MLLT10  PICALM (11q14.2) MLLT10 (10p12.31)
 
Disease databaset(10;11)(p13;q21) PICALM/MLLT10
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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