TY - JOUR
T1 - NUP98 rearrangements in hematopoietic malignancies
T2 - A study of the Groupe Francophone de Cytogénétique Hématologique
AU - Romana, Serge
AU - Radford-Weiss, I.
AU - Abdelali, R. Ben
AU - Schluth, C.
AU - Petit, A.
AU - Dastugue, N.
AU - Talmant, P.
AU - Bilhou-Nabera, C.
AU - Mugneret, F.
AU - Lafage-Pochitaloff, M.
AU - Mozziconacci, M. J.
AU - Andrieu, J.
AU - Lai, J. L.
AU - Terre, C.
AU - Rack, K.
AU - Cornillet-Lefebvre, P.
AU - Luquet, I.
AU - Nadal, N.
AU - Nguyen-Khac, F.
AU - Perot, C.
AU - Van den Akker, J.
AU - Fert-Ferrer, S.
AU - Cabrol, C.
AU - Charrin, C.
AU - Tigaud, I.
AU - Poirel, H.
AU - Vekemans, M.
AU - Bernard, O. A.
AU - Berger, R.
N1 - Funding Information:
We gratefully acknowledge S Nusbaum and F Poulain for excelent technical assistance. This work was supported by the Ligue contre le cancer (labeled team: SPR, IRW, AP, C.S, FNK, OAB, RB).
PY - 2006/1/1
Y1 - 2006/1/1
N2 - The NUP98 gene is fused with 19 different partner genes in various human hematopoietic malignancies. In order to gain additional clinico-hematological data and to identify new partners of NUP98, the Groupe Francophone de Cytogénétique Hématologique (GFCH) collected cases of hematological malignancies where a 11p15 rearrangement was detected. Fluorescence in situ hybridization (FISH) analysis showed that 35% of these patients (23/66) carried a rearrangement of the NUP98 locus. Genes of the HOXA cluster and the nuclear-receptor set domain (NSD) genes were frequently fused to NUP98, mainly in de novo myeloid malignancies whereas the DDX10 and TOP1 genes were equally rearranged in de novo and in therapy-related myeloid proliferations. Involvement of ADD3 and C6ORF80 genes were detected, respectively, in myeloid disorders and in T-cell acute lymphoblastic leukemia (T-ALL), whereas the RAP1GDS1 gene was fused to NUP98 in T-ALL. Three new chromosomal breakpoints: 3q22.1, 7p15 (in a localization distinct from the HOXA locus) and Xq28 were detected in rearrangements with the NUP98 gene locus. The present study as well as a review of the 73 cases previously reported in the literature allowed us to delineate some chromosomal, clinical and molecular features of patients carrying a NUP98 gene rearrangements.
AB - The NUP98 gene is fused with 19 different partner genes in various human hematopoietic malignancies. In order to gain additional clinico-hematological data and to identify new partners of NUP98, the Groupe Francophone de Cytogénétique Hématologique (GFCH) collected cases of hematological malignancies where a 11p15 rearrangement was detected. Fluorescence in situ hybridization (FISH) analysis showed that 35% of these patients (23/66) carried a rearrangement of the NUP98 locus. Genes of the HOXA cluster and the nuclear-receptor set domain (NSD) genes were frequently fused to NUP98, mainly in de novo myeloid malignancies whereas the DDX10 and TOP1 genes were equally rearranged in de novo and in therapy-related myeloid proliferations. Involvement of ADD3 and C6ORF80 genes were detected, respectively, in myeloid disorders and in T-cell acute lymphoblastic leukemia (T-ALL), whereas the RAP1GDS1 gene was fused to NUP98 in T-ALL. Three new chromosomal breakpoints: 3q22.1, 7p15 (in a localization distinct from the HOXA locus) and Xq28 were detected in rearrangements with the NUP98 gene locus. The present study as well as a review of the 73 cases previously reported in the literature allowed us to delineate some chromosomal, clinical and molecular features of patients carrying a NUP98 gene rearrangements.
KW - Chromosome 11p15
KW - FISH
KW - Leukemia
KW - NUP98
UR - http://www.scopus.com/inward/record.url?scp=33646484524&partnerID=8YFLogxK
U2 - 10.1038/sj.leu.2404130
DO - 10.1038/sj.leu.2404130
M3 - Article
C2 - 16467868
AN - SCOPUS:33646484524
SN - 0887-6924
VL - 20
SP - 696
EP - 706
JO - Leukemia
JF - Leukemia
IS - 4
ER -