Written | 1998-01 | Jean-Loup Huret |
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France |
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) |
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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 Name | MLLT10 (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 Name | PICALM (clathrin assembly lymphoid myeloid leukemia gene) |
Location | 11q14.2 |
Protein | 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 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)(p12;q21) PICALM/MLLT10 | |
External links |
Mitelman database | t(10;11)(p12;q21) |
arrayMap (UZH-SIB Zurich) | Topo ( C42) Morph ( 9811/3) - [auto + random 100 samples .. if exist ] [tabulated segments] |
arrayMap (UZH-SIB Zurich) | Topo ( C42) Morph ( 9837/3) - [auto + random 100 samples .. if exist ] [tabulated segments] |
arrayMap (UZH-SIB Zurich) | Topo ( C42) Morph ( 9861/3) - [auto + random 100 samples .. if exist ] [tabulated segments] |
Mitelman database | PICALM/MLLT10 [MCList] PICALM (11q14.2) MLLT10 (10p12.31) |
TCGA_Fusion | PICALM/MLLT10 [LAML] PICALM (11q14.2) MLLT10 (10p12.31) |
TICdb | PICALM/MLLT10 PICALM (11q14.2) MLLT10 (10p12.31) |
REVIEW articles | automatic search in PubMed |
Last year articles | automatic search in PubMed |
All articles | automatic search in PubMed |
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