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Multicenter Study
. 2009 Feb;23(2):323-31.
doi: 10.1038/leu.2008.312. Epub 2008 Nov 20.

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

H A Poirel  1 , M S Cairo, N A Heerema, J Swansbury, A Aupérin, E Launay, W G Sanger, P Talley, S L Perkins, M Raphaël, K McCarthy, R Sposto, M Gerrard, A Bernheim, C Patte; FAB/LMB 96 International Study Committee
Collaborators, Affiliations
Multicenter Study

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

H A Poirel et al. Leukemia. 2009 Feb.

Abstract

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.

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Figures

Figure 1
Figure 1. Distribution of cytogenetic abnormalities in FAB/LMB 96 Study stratified by histological subtypes Burkitt Lymphoma (BL), Burkitt Like Lymphoma (BLL) and Diffuse Large B-Cell Lymphoma (DLBCL)
R8q24, dup(1q), del(6q), der(11q), +12, ploidy and complexity are helpful in the discrimination between BL and DLBCL. BLL exhibit an intermediary pattern.
Figure 2
Figure 2. Probability of EFS by Kaplan-Meier method in children with B-NHL treated on FAB/LMB 96 on the whole population (N=238)
Figure 2A: EFS with and without rearranged 8q24 cytogenetic abnormality Figure 2B: EFS with and without del(13q) cytogenetic abnormality Figure 2C: EFS with and without +7q cytogenetic abnormality Figure 2D: EFS with and without a complex karyotype (>3 cytogenetic abnormalities)
Figure 3
Figure 3. Probability of EFS by Kaplen-Meier method in children with B-NHL treated on FAB/LMB 96 according to the main morphologic entities BL (N=182) and DLBCL (N=30)
Figure 3A: EFS with and without rearranged 8q24 cytogenetic abnormality Figure 3B: EFS with and without del(13q) cytogenetic abnormality Figure 3C: EFS with and without +7q cytogenetic abnormality Figure 3D: EFS with and without a complex karyotype (>3 cytogenetic abnormalities) While del(13q), +7q and the complexity altered the prognosis of BL and DLBCL in the same proportion, the adverse effect of R8q24 is only detected in DLBCL.
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References

    1. Patte C, Auperin A, Gerrard M, Michon J, Pinkerson CR, Sposto R, et al. Results of the randomized international FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin’s lymphoma in children and adolescents: it is possible to reduce treatment for the early responding patients. Blood. 2007;109:2773–2780. - PMC - PubMed
    1. Cairo MS, Gerrard M, Sposto R, Auperin A, Pinkerson CR, Michon J, et al. Results of a randomized international study of high risk central nervous system B-non-Hodgkin’s lymphoma and B-acute lymphoblastic leukemia in children and adolescents. Blood. 2007;109:2736–2743. - PMC - PubMed
    1. Gerrard M, Cairo MS, Weston C, Auperin A, Pinkerton R, Lambilliote A, et al. Excellent survival following two courses of COPAD chemotherapy in children and adolescents with resected localized B-cell Non-Hodgkin’s Lymphoma: Results of the FAB/LMB 96 International Study. Br J Haematol. 2008;141:840–7. - PubMed
    1. Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84:1361–1392. - PubMed
    1. Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Pathology & genetics of tumours of haematopoietic and lymphoid tissues. Lyon: IARCPress; 2001. World Health Organization Classification of tumors; pp. 181–184.

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