| 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) |
 | |
| 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)(p13;q21) PICALM/MLLT10 | |
| External links |
| REVIEW articles | automatic search in PubMed |
| Last year articles | automatic search in PubMed |
| All articles | automatic search in PubMed |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon Sep 18 17:19:59 CEST 2017 |
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