دورية أكاديمية
Mutated WT1, FLT3-ITD, and NUP98-NSD1 Fusion in Various Combinations Define a Poor Prognostic Group in Pediatric Acute Myeloid Leukemia
| العنوان: | Mutated WT1, FLT3-ITD, and NUP98-NSD1 Fusion in Various Combinations Define a Poor Prognostic Group in Pediatric Acute Myeloid Leukemia |
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| المؤلفون: | Naghmeh Niktoreh, Christiane Walter, Martin Zimmermann, Christine von Neuhoff, Nils von Neuhoff, Mareike Rasche, Katharina Waack, Ursula Creutzig, Helmut Hanenberg, Dirk Reinhardt |
| المصدر: | Journal of Oncology, Vol 2019 (2019) |
| بيانات النشر: | Hindawi Limited |
| سنة النشر: | 2019 |
| المجموعة: | Directory of Open Access Journals: DOAJ Articles |
| مصطلحات موضوعية: | Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282 |
| الوصف: | Acute myeloid leukemia is a life-threatening malignancy in children and adolescents treated predominantly by risk-adapted intensive chemotherapy that is partly supported by allogeneic stem cell transplantation. Mutations in the WT1 gene and NUP98-NSD1 fusion are predictors of poor survival outcome/prognosis that frequently occur in combination with internal tandem duplications of the juxta-membrane domain of FLT3 (FLT3-ITD). To re-evaluate the effect of these factors in contemporary protocols, 353 patients (<18 years) treated in Germany with AML-BFM treatment protocols between 2004 and 2017 were included. Presence of mutated WT1 and FLT3-ITD in blasts (n=19) resulted in low 3-year event-free survival of 29% and overall survival of 33% compared to rates of 45-63% and 67-87% in patients with only one (only FLT3-ITD; n=33, only WT1 mutation; n=29) or none of these mutations (n=272). Including NUP98-NSD1 and high allelic ratio (AR) of FLT3-ITD (AR ≥0.4) in the analysis revealed very poor outcomes for patients with co-occurrence of all three factors or any of double combinations. All these patients (n=15) experienced events and the probability of overall survival was low (27%). We conclude that co-occurrence of WT1 mutation, NUP98-NSD1, and FLT3-ITD with an AR ≥0.4 as triple or double mutations still predicts dismal response to contemporary first- and second-line treatment for pediatric acute myeloid leukemia. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 1687-8450 1687-8469 |
| العلاقة: | http://dx.doi.org/10.1155/2019/1609128Test; https://doaj.org/toc/1687-8450Test; https://doaj.org/toc/1687-8469Test; https://doaj.org/article/08bdb044c5a74a76a798756174f9434bTest |
| DOI: | 10.1155/2019/1609128 |
| الإتاحة: | https://doi.org/10.1155/2019/1609128Test https://doaj.org/article/08bdb044c5a74a76a798756174f9434bTest |
| رقم الانضمام: | edsbas.179757DF |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 16878450 16878469 |
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| DOI: | 10.1155/2019/1609128 |