Atlas of Genetics and Cytogenetics in Oncology and Haematology
t(10;11)(p13;q21)
| Identity |
| 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) R-banding (left) 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 ANLL and/or ANLL with T-cell markers, or B-cell ALL PHENOTYPE_STEM_CELL_ORIGIN a myelomonocytic/T- cell common progenitor may be involved; FAB: L1/L2 |
| 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 | AF10 |
| Location | 10p12 |
| Dna / Rna | 5' telomeric --> 3' centromeric orientation |
| Protein | contains 3 Zn fingezrs and a leucine zipper; nuclear localisation; transcription factor |
| Gene Name | CALM |
| Location | 11q14-21 |
| 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 |
| External links |
| Other database | t(10;11)(p13;q21) | Mitelman database (CGAP - NCBI) | |
| Other database | t(10;11)(p13;q21) | CancerChromosomes (NCBI) |
| Bibliography |
| t(10;11)(p13-14;q14-21): a new recurrent translocation in T-cell acute lymphoblastic leukemias. Groupe Franˆßais de Cytogˆ©nˆ©tique Hˆ©matologique (GFCH). |
| Genes, chromosomes & cancer. 1991 ; 3 (6) : 411-415. |
| PMID 1777411 |
| 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 |
| 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 |
| Contributor(s) |
| Written | 01-1998 | Jean-Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . t(10;11)(p13;q21). Atlas Genet Cytogenet Oncol Haematol. January 1998 . URL : http://AtlasGeneticsOncology.org/Anomalies/t1011bis.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon May 12 18:12:54 2008 |
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