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Specific cytogenetic abnormalities are associated with a significantly inferior outcome in children and adolescents with mature B-cell non-Hodgkin's lymphoma: results of the FAB/LMB 96 international study

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Poirel, Hélène [UCL] Cairo, M. S. Heerema, N. A. Swansbury, J. Auperin, A. Patte, C.

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.

Document type Communication à un colloque (Conference Paper) – Présentation orale avec comité de sélection
Publication date 2009
Language Anglais
Conference "9th International Conference on Malignant Lymphoma", Lugano (Switzerland) (Jun 09-11, 2005)
Journal information "Leukemia" - Vol. 23, no. 2, p. 323-331 (2009)
Peer reviewed yes
issn 0887-6924
e-issn 1476-5551
Publisher Nature Publishing Group (London)
Affiliations UCL - Cliniques universitaires Saint-Luc
UCL - MD/BICL - Département de biochimie et de biologie cellulaire
Keywords Childhood Mature B-cell Lymphoma ; Burkitt Lymphoma ; Dlbcl ; Cytogenetics ; Prognosis
Links
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Bibliographic reference Poirel, Hélène ; Cairo, M. S. ; Heerema, N. A. ; Swansbury, J. ; Auperin, A. ; et. al. Specific cytogenetic abnormalities are associated with a significantly inferior outcome in children and adolescents with mature B-cell non-Hodgkin's lymphoma: results of the FAB/LMB 96 international study.9th International Conference on Malignant Lymphoma (Lugano (Switzerland), Jun 09-11, 2005). In: Leukemia, Vol. 23, no. 2, p. 323-331 (2009)
Permanent URL http://hdl.handle.net/2078.1/59035