Journalartikel
Autorenliste: Bonn, Bettina R.; Rohde, Marius; Zimmermann, Martin; Krieger, David; Oschlies, Ilske; Niggli, Felix; Wrobel, Grazyna; Attarbaschi, Andishe; Escherich, Gabriele; Klapper, Wolfram; Reiter, Alfred; Burkhardt, Birgit
Jahr der Veröffentlichung: 2013
Seiten: 3153-3160
Zeitschrift: Blood
Bandnummer: 121
Heftnummer: 16
ISSN: 0006-4971
eISSN: 1528-0020
Open Access Status: Green
DOI Link: https://doi.org/10.1182/blood-2012-12-474148
Verlag: American Society of Hematology (ASH Publications)
Abstract:
Probability of event-free survival (pEFS) in pediatric T-cell lymphoblastic lymphoma is about 80%, whereas survival in relapsed patients is very poor. No stratification criteria have been established so far. Recently, activating NOTCH1 mutations were reported to be associated with favorable prognosis, and loss of heterozygosity at chromosome 6q (LOH6q) was reported to be associated with increased relapse risk. The current project was intended to evaluate the prognostic effect of these markers. Mutations in hot spots of NOTCH1 and FBXW7 were analyzed in 116 patients. Concerning LOH6q status, 118 patients were investigated, using microsatellite marker analysis, in addition to an earlier reported cohort of 99 available patients. Ninety-two cases were evaluable for both analyses. All patients were treated with T-cell lymphoblastic lymphoma-Berlin-Frankfurt-Munster group (BFM)-type treatment. LOH6q was observed in 12% of patients (25/217) and associated with unfavorable prognosis (pEFS 27% +/- 9% vs 86% +/- 3%; P < .0001). In 60% (70/116) of the patients, NOTCH1 mutations were detected and associated with favorable prognosis (pEFS 84% +/- 5% vs 66% +/- 7%; P = .021). Interestingly, NOTCH1 mutations were rarely observed in patients with LOH in 6q16. Both prognostic markers will be used as stratification criteria in coming Non-Hodgkin Lymphoma-BFM trials.
Zitierstile
Harvard-Zitierstil: Bonn, B., Rohde, M., Zimmermann, M., Krieger, D., Oschlies, I., Niggli, F., et al. (2013) Incidence and prognostic relevance of genetic variations in T-cell lymphoblastic lymphoma in childhood and adolescence, Blood, 121(16), pp. 3153-3160. https://doi.org/10.1182/blood-2012-12-474148
APA-Zitierstil: Bonn, B., Rohde, M., Zimmermann, M., Krieger, D., Oschlies, I., Niggli, F., Wrobel, G., Attarbaschi, A., Escherich, G., Klapper, W., Reiter, A., & Burkhardt, B. (2013). Incidence and prognostic relevance of genetic variations in T-cell lymphoblastic lymphoma in childhood and adolescence. Blood. 121(16), 3153-3160. https://doi.org/10.1182/blood-2012-12-474148
Schlagwörter
CYTOGENETIC ABNORMALITIES; FBXW7 MUTATIONS; HETEROZYGOSITY; HIGH-DOSE METHOTREXATE; LEUKEMIA; NOTCH1 MUTATIONS
