TY - JOUR
T1 - Specific cytogenetic abnormalities are associated with a significantly inferior outcome in children and adolescents with mature B-cell non-Hodgkin's lymphoma
T2 - Results of the FAB/LMB 96 international study
AU - Poirel, H. A.
AU - Cairo, M. S.
AU - Heerema, N. A.
AU - Swansbury, J.
AU - Aupérin, A.
AU - Launay, E.
AU - Sanger, W. G.
AU - Talley, P.
AU - Perkins, S. L.
AU - Raphaël, M.
AU - McCarthy, K.
AU - Sposto, R.
AU - Gerrard, M.
AU - Bernheim, A.
AU - Patte, C.
N1 - Funding Information:
We thank the other members of the international morphological review panel: MJ Terrier-Lacombe, C Bayle, B Felman (SFOP), M Lones (CCG) and A Wotherspoon UKCCSG). We further thank the data managers of the SFOP, CCG and UKCCSG cooperative groups, Virginia Davenport and Lauren Harrison for their active and helpful support of COG part of the study, and all the investigators who treated the patients and participated in the study. This work was supported by grants from the Division of Cancer Treatment, National Cancer Institute, National Institutes of Health, Department of Health and Human Services (COG); Cancer Research Campaign, (UKCSSG), Association pour la Recherche contre le Cancer), La Ligue Nationale Contre le Cancer, Institut Gustave Roussy (SFOP) and COG Grant CA 98543. A complete listing of grant support for research conducted by CCG and POG before initiation of the COG grant in 2003 is available online at: http://www.childrensoncologygroup.org/admin/grantinfo.htm.
PY - 2009/1/1
Y1 - 2009/1/1
N2 - Clinical studies showed that advanced stage, high LDH, poor response to reduction therapy and combined bone marrow and central nervous system disease are significantly associated with a decreased event-free survival (EFS) in pediatric mature B-cell non-Hodgkin's lymphoma (B-NHL) treated on FAB/LMB96. Although rearranged MYC/8q24 (R8q24) is characteristic of Burkitt lymphoma (BL), little information is available on other cytogenetic abnormalities and their prognostic importance. We performed an international review of 238 abnormal karyotypes in childhood mature B-NHL treated on FAB/LMB96: 76% BL, 8% Burkitt-like lymphoma, 13% diffuse large B-cell lymphoma (DLBCL). The main BL R8q24-associated chromosomal aberrations were +1q (29%), +7q and del(13q) (14% each). The DLBCL appeared heterogeneous and more complex. Incidence of R8q24 (34%) was higher than reported in adult DLBCL. The prognostic value of cytogenetic abnormalities on EFS was studied by Cox model controlling for the known risk factors: R8q24, +7q and del(13q) were independently associated with a significant inferior EFS (hazard ratio: 6.1 (P=0.030), 2.5 (P=0.015) and 4.0 (P=0.0003), respectively). The adverse prognosis of R8q24 was observed only in DLBCL, whereas del(13q) and +7q had a similar effect in DLBCL and BL. These results emphasize the significant biological heterogeneity and the development of cytogenetic risk-adapted therapy in childhood mature B-NHL.
AB - Clinical studies showed that advanced stage, high LDH, poor response to reduction therapy and combined bone marrow and central nervous system disease are significantly associated with a decreased event-free survival (EFS) in pediatric mature B-cell non-Hodgkin's lymphoma (B-NHL) treated on FAB/LMB96. Although rearranged MYC/8q24 (R8q24) is characteristic of Burkitt lymphoma (BL), little information is available on other cytogenetic abnormalities and their prognostic importance. We performed an international review of 238 abnormal karyotypes in childhood mature B-NHL treated on FAB/LMB96: 76% BL, 8% Burkitt-like lymphoma, 13% diffuse large B-cell lymphoma (DLBCL). The main BL R8q24-associated chromosomal aberrations were +1q (29%), +7q and del(13q) (14% each). The DLBCL appeared heterogeneous and more complex. Incidence of R8q24 (34%) was higher than reported in adult DLBCL. The prognostic value of cytogenetic abnormalities on EFS was studied by Cox model controlling for the known risk factors: R8q24, +7q and del(13q) were independently associated with a significant inferior EFS (hazard ratio: 6.1 (P=0.030), 2.5 (P=0.015) and 4.0 (P=0.0003), respectively). The adverse prognosis of R8q24 was observed only in DLBCL, whereas del(13q) and +7q had a similar effect in DLBCL and BL. These results emphasize the significant biological heterogeneity and the development of cytogenetic risk-adapted therapy in childhood mature B-NHL.
UR - http://www.scopus.com/inward/record.url?scp=60149085845&partnerID=8YFLogxK
U2 - 10.1038/leu.2008.312
DO - 10.1038/leu.2008.312
M3 - Article
C2 - 19020548
AN - SCOPUS:60149085845
SN - 0887-6924
VL - 23
SP - 323
EP - 331
JO - Leukemia
JF - Leukemia
IS - 2
ER -