Atlas of Genetics and Cytogenetics in Oncology and Haematology
+13,+13 or tetrasomy 13
| Identity |
 | |
| GTG- banded (left) and RHG-banded (right) metaphases on blood cells showing the isolated tetrasomy 13. In the present case, the specific 13ps+ polymorphism of one of the chromosomes 13 revealed that tetrasomy resulted in the triplication of the same parental chromosome 13 rather than a double-duplication mechanism. | |
| Clinics and Pathology |
| Disease | Acute myeloid leukaemia, poorly differenciated (AML-M0). |
| Epidemiology | Only 4 cases of primary acquired isolated tetrasomy have been described in patients with undifferentiated acute myeloid leukemia. |
| Prognosis | The possibility that isolated tetrasomy 13 may represent an independent poor prognostic factor could be suggested by the poor outcome under therapy in our patients and those reported previously. However, responses to intensive chemotherapy in older patients (under 60 year of age) are lower than with younger patients, and all described cases of isolated tetrasomy 13 occurred in elderly subjects. |
| Genetics |
| Two candidate genes mapped on chromosome 13 whose deregulated function might contribute to the development of transformation of undifferentiated myeloid cells are FLT1 and Rb1. However, their involvement in acute leukemia with trisomy 13 / tetrasomy 13 have to be determined, and the mechanism whereby the increased gene dose alone or in association with other additional mutation(s) confers neoplastic potential of undifferentiated phenotype is unknown. |
| Cytogenetics |
| Note | Tetrasomy 13 can occur in different cases of acute leukemia with trisomy 13 as the primary cytogenetic abnormality, or can be associated with additional abnormalities following transformation. |
| External links |
| Other database | +13,+13 or tetrasomy 13 | Mitelman database (CGAP - 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 |
| Trisomy 13: a new recurring chromosome abnormality in acute leukemia. |
| Dhner H, Arthur DC, Ball ED, Sobol RE, Davey FR, Lawrence D, Gordon L, Patil SR, Surana RB, Testa JR |
| Blood. 1990 ; 76 (8) : 1614-1621. |
| PMID 1698482 |
| Trisomy/tetrasomy 13 in seven cases of acute leukemia. |
| Sreekantaiah C, Baer MR, Morgan S, Isaacs JD, Miller KB, Sandberg AA |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1990 ; 4 (11) : 781-785. |
| PMID 2232892 |
| Tetrasomy 13 as the sole cytogenetic abnormality in acute myeloid leukemia M1 without maturation. |
| McGrattan P, Alexander HD, Humphreys MW, Kettle PJ |
| Cancer genetics and cytogenetics. 2002 ; 135 (2) : 192-195. |
| PMID 12127406 |
| Isolated tetrasomy 13: a fifth case report of a rare chromosome abnormality associated with poorly differentiated acute myeloid leukemia. |
| Roche-Lestienne C, Richebourg S, La JL, Andrieux J, Soenen-Cornu V, Geffroy S |
| Cancer genetics and cytogenetics. 2006 ; 168 (2) : 181-182. |
| PMID 16843114 |
| Contributor(s) |
| Written | 09-2006 | Catherine Roche-Lestienne |
| Citation |
| This paper should be referenced as such : |
| Roche-Lestienne C . +13,+13 or tetrasomy 13. Atlas Genet Cytogenet Oncol Haematol. Septem
ber 2006 . URL : http://AtlasGeneticsOncology.org/Genes/Tetra13ID1260.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Sat Dec 6 18:01:38 2008 |
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