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+13,+13 or tetrasomy 13

Written2006-09Catherine Roche-Lestienne
(1) : Laboratoire de Génétique Médicale, Hôpital Jeanne de Flandre - CHRU de Lille, France; (2) : Département d'Hématologie, Université Catholique de Lille, France; (3) : Laboratoire d'Hématologie A, Hôpital Calmette - CHRU de Lille, France

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Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9861/3 AML with mutated NPM1; AML with mutated CEBPA; Acute myeloid leukaemia, NOS
Atlas_Id 1260
 
  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.

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.
Döhner 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
 
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, Laï JL, Andrieux J, Soenen-Cornu V, Geffroy S
Cancer genetics and cytogenetics. 2006 ; 168 (2) : 181-182.
PMID 16843114
 
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
 

Citation

This paper should be referenced as such :
Roche-Lestienne, C
+13,+13 or tetrasomy 13
Atlas Genet Cytogenet Oncol Haematol. 2007;11(1):38-39.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/Tetra13ID1260.html


External links

arrayMapTopo ( C42) Morph ( 9861/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease database+13,+13 or tetrasomy 13
REVIEW articlesautomatic search in PubMed
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