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Atlas of Genetics and Cytogenetics
in Oncology and Haematology
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Leukaemia Section
Mini Review
+13,+13 or tetrasomy 13
Catherine Roche-Lestienne
Laboratoire de Génétique Médicale, Hôpital Jeanne de Flandre - CHRU de Lille, France
Published in Atlas Database: September 2006
Online updated version: http://AtlasGeneticsOncology.org/Anomalies/Tetra13ID1260.html
DOI: 10.4267/2042/38386
This work is licensed under a Creative Commons Attribution-Non-commercial-No Derivative Works 2.0 France Licence.
© 2007 Atlas of Genetics and Cytogenetics in Oncology and Haematology
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.
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.
Clinics and pathology
Disease
Acute myeloid leukaemia, poorly differenciated (AMLM0).
Genetics
Epidemiology
Note: 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.
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,
Atlas Genet Cytogenet Oncol Haematol. 2007;11(1)
38
+13,+13 or tetrasomy 13
Roche-Lestienne C
Sreekantaiah C, Baer MR, Morgan S, Isaacs JD, Miller KB,
Sandberg AA. Trisomy/tetrasomy 13 in seven cases of acute
leukemia. Leukemia 1990;4:781-795.
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.
McGrattan P, Alexander HD, Humphreys MW, Kettle PJ.
Tetrasomy 13 as the sole cytogenetic abnormality in acute
myeloid leukemia M1 without maturation. Cancer Genet
Cytogenet 2002;135:192-195.
Roche-Lestienne C, Soenen V, Richebourg S, Geffroy S, Laï
JL, Andrieux J. Isolated tetrasomy 13: a fifth case report of a
rare chromosome abnormality associated with poorly
differentiated acute myeloid leukemia. Cancer Genet.
Cytogenet 2006;168:181-182.
References
Döhner H, Arthur DC, Ball ED, Sobol RE, Davey FR, Lawrence
D, Gordon L, Patil SR, Surana RB, Testa JR, Verma RS,
Schiffer CA, Wurster-Hill DH, Bloomfield CD. Trisomy 13: a
new recurring chromosome abnormality in acute leukemia.
Blood 1990;76:1614-1621.
Atlas Genet Cytogenet Oncol Haematol. 2007;11(1)
This article should be referenced as such:
Roche-Lestienne C. +13,+13 or tetrasomy 13. Atlas Genet
Cytogenet Oncol Haematol.2007;11(1):38-39.
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