| Written | 2001-10 | 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 | 9807/3 Mixed phenotype acute leukaemia with t(v;11q23); MLL rearranged |
| ICD-Morpho | 9813/3 B lymphoblastic leukaemia/lymphoma with t(v;11q23); MLL rearranged |
| ICD-Morpho | 9920/3 Therapy-related myeloid neoplasms |
| Atlas_Id | 1223 |
| Clinics and Pathology |
| Disease | treatment related acute myeloid leukemia (t-AML) so far |
| Epidemiology | only 1 case available |
| Clinics | a 41 yr old female patient with a previous history of non Hodgkin lymphoma treated withtopoisomerase II inhibitors 3 yrs earlier; she developped a M5a t-AML and died 10 days after diagnosis |
| Cytogenetics |
| Additional anomalies | a complex karyotype was found in the only available case, with +8 and other anomalies. |
| Genes involved and Proteins |
| Gene Name | LPP (lipoma preferred partner) |
| Location | 3q28 |
| Protein | LPP is a member of the LIM protein gene family, containing a leucin-zipper motif in its amino-terminal region and three LIM domains in its carboxy-terminal region |
| Gene Name | KMT2A (myeloid/lymphoid or mixed lineage leukemia) |
| Location | 11q23.3 |
| Protein | contains two DNA binding motifs (a AT hook, and Zinc fingers), a DNA methyl transferase motif, a bromodomain; transcriptional regulatory factor involved in maintenance of Hox gene expression |
| Result of the chromosomal anomaly |
| Description | 5' MLL including the 8 first exons fused to the 3 last 3' exons (exons 9 to 11) of LPP. |
| Transcript | the reciprocal 5'MLPP-3'MLL is also transcribed |
| Description | N-term AT hook and DNA methyltransferase from MLL fused to the 2 last C-term LIM domains of LPP |
| 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 |
| Human LPP gene is fused to MLL in a secondary acute leukemia with a t(3;11) (q28;q23). |
| Dahéron L, Veinstein A, Brizard F, Drabkin H, Lacotte L, Guilhot F, Larsen CJ, Brizard A, Roche J |
| Genes, chromosomes & cancer. 2001 ; 31 (4) : 382-389. |
| PMID 11433529 |
| Citation |
| This paper should be referenced as such : |
| Huret, JL |
| t(3;11)(q28;q23) |
| Atlas Genet Cytogenet Oncol Haematol. 2002;6(1):39-39. |
| Free journal version : [ pdf ] [ DOI ] |
| On line version : http://AtlasGeneticsOncology.org/Anomalies/t0311q28q23ID1223.html |
| Translocations implicated (Data extracted from papers in the Atlas) |
| t(3;11)(q28;q23) KMT2A/LPP | |
| 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 Aug 12 17:00:02 CEST 2019 |
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