| Clinics and Pathology |
| Disease | Acute myeloid leukemia (AML) |
| Phenotype / cell stem origin | Described in subtypes AML-M2, -M4 and -M5. Cell lineage dysplasia may be associated. |
| Epidemiology | Less than 10 cases described, mainly adults and one child. |
| Prognosis | Undetermined, possibly intermediate. |
| Cytogenetics |
| Cytogenetics Morphological | Easy to detect, evident 10q- and 11q+ derivatives. |
| Cytogenetics Molecular | Commercial dual color MLL FISH probes are splitted by the translocation. 10q22 breakpoint may be detected with RP11-119F7 BAC probe. |
| Additional anomalies | Sole anomaly in half published cases, complex karyotype in others. |
| Genes involved and Proteins |
| Gene Name | MLL |
| Location | 11q23 |
| Note | MLL (mixed-lineage leukemia or myeloid-lymphoid leukaemia) is also called ALL-1 or HRX |
| Dna / Rna | 36 exons, multiple transcripts 13-15 kb. |
| Protein | 430 kDA, contains two DNA binding motifs (a AT hook and a CXXC domain), a DNA methyl transferase motif, a bromodomain; transcriptional regulatory factor involved in maintenance of Hox gene expression during embryogenesis and during the process of haematopoietic progenitors expansion and differentiation. |
| Gene Name | CXXC6 |
| Location | 10q22 |
| Note | CXXC6 ( CXXC finger 6 ) is also called LCX (leukemia-associated protein with a CXXC domain) or TET1 |
| Dna / Rna | 8497 bp representing the whole coding sequence. At least 12 exons. Contains 3 bipartite nuclear localization sites, 1 alpha helice coiled-coil region and 1 cysteine rich domain with high level homology with a CXXC DNA binding site. |
| Protein | Predicted size of 2136 amino acids, expression restricted to some fetal tissues, mainly lung, heart and brain; not expressed in hematopoietic tissues, except in spleen; unknown function. |
| Result of the chromosomal anomaly |
| Description | Breakpoint within MLL intron 6 and LCX intron 8; MLL exon 8 is fused in frame with LCX exon 9 and transcripts from the 5' MLL-LCX 3' fusion gene on der(11) are expressed; transcripts from the 5' LCX-MLL3' counterpart are not detected. |
| Description | Predicted molecular weight of 204.4 kDa. |
| Oncogenesis | Unknown; the alpha helice coiled-coil region retained at the COOH extremity might be involved in the leukemogenesis. |
| External links |
| Other database | t(10;11)(q22;q23) | Mitelman database (CGAP - NCBI) | |
| Other database | t(10;11)(q22;q23) | CancerChromosomes (NCBI) |
| To be noted |
| Additional cases are needed to delineate the epidemiology of this rare entity: you are welcome to submit a paper to our new Case Report section. |
| Bibliography |
| Involvement of MLL gene in a t(10;11)(q22;q23) and a t(8;11)(q24;q23) identified by fluorescence in situ hybridization. |
| Aventˆ‚n A, La Starza R, Martˆ‚nez C, Wlodarska I, Boogaerts M, Van den Berghe H, Mecucci C |
| Cancer genetics and cytogenetics. 1999 ; 108 (1) : 48-52. |
| PMID 9973924 |
| LCX, leukemia-associated protein with a CXXC domain, is fused to MLL in acute myeloid leukemia with trilineage dysplasia having t(10;11)(q22;q23). |
| Ono R, Taki T, Taketani T, Taniwaki M, Kobayashi H, Hayashi Y |
| Cancer research. 2002 ; 62 (14) : 4075-4080. |
| PMID 12124344 |
| TET1, a member of a novel protein family, is fused to MLL in acute myeloid leukemia containing the t(10;11)(q22;q23). |
| Lorsbach RB, Moore J, Mathew S, Raimondi SC, Mukatira ST, Downing JR |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 2003 ; 17 (3) : 637-641. |
| PMID 12646957 |
| Identification and characterization of human CXXC10 gene in silico. |
| Katoh M, Katoh M |
| International journal of oncology. 2004 ; 25 (4) : 1193-1199. |
| PMID 15375572 |
| Contributor(s) |
| Written | 02-2008 | Franck Viguié |
| Laboratoire de Cytogénétique - Service d'Hématologie Biologique, Hopital Hotel-Dieu - 75181 Paris Cedex 04, France |
| Citation |
| This paper should be referenced as such : |
| Viguié F . t(10;11)(q22;q23). Atlas Genet Cytogenet Oncol Haematol. February 2008 . URL : http://AtlasGeneticsOncology.org/Genes/t1011q22q23ID1410.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon Aug 11 21:20:42 2008 |
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