Prognostic Role of Minimal Disseminated Disease and NOTCH1/FBXW7 Mutational Status in Children with Lymphoblastic Lymphoma: The AIEOP Experience

Autor:	Barbara Michielotto, Marta Pillon, Veronica Maria Folsi, Lara Mussolin, Emanuele S.G. d'Amore, Paola Muggeo, Alessandra Biffi, Elisa Carraro, Federica Lovisa, Luciana Vinti, Marco Pizzi, M. Piglione, Carlotta C. Damanti, Cristiano Pasin, Daniela Onofrillo, Elena Varotto, Anna Garbin, Salvatore Buffardi, Barbara Buldini, Ilaria Gallingani
Jazyk:	angličtina
Rok vydání:	2021
Předmět:	Oncology medicine.medical_specialty Medicine (General) Clinical Biochemistry NOTCH1/FBXW7 child lymphoblastic lymphoma minimal disseminated disease Article R5-920 Internal medicine Medicine Mutational status Stage (cooking) business.industry Intensive treatment Significant difference Lymphoblastic lymphoma medicine.disease Peripheral blood medicine.anatomical_structure Minimal Disseminated Disease Bone marrow business
Zdroj:	Diagnostics Diagnostics, Vol 11, Iss 1594, p 1594 (2021) Volume 11 Issue 9
ISSN:	2075-4418
Popis:	NOTCH1/FBXW7 (N/F) mutational status at diagnosis is employed for T-cell lymphoblastic lymphoma (T-LBL) patients’ stratification in the international protocol LBL 2018. Our aim was to validate the prognostic role of Minimal Disseminated Disease (MDD) alone and in combination with N/F mutational status in a large retrospective series of LBL pediatric patients. MDD was analyzed in 132 bone marrow and/or peripheral blood samples by flow cytometry. Mutations in N/F genes were analyzed on 58 T-LBL tumor biopsies. Using the previously established cut-off of 3%, the four-year progression-free survival (PFS) was 57% for stage I–III patients with MDD ≥ 3% versus 80% for patients with MDD inferior to cut-off (p = 0.068). We found a significant worsening in the four-year PFS for nonmutated (51 ± 12%) compared to mutated patients (100%, p = 0.0013). Combining MDD and N/F mutational status in a subgroup of available cases, we found a statistically significant difference in the four-year PFS for different risk groups (p = 0.0012). Overall, our results demonstrate that N/F mutational status has a more relevant prognostic value than MDD at diagnosis. However, the combination of N/F mutations with MDD analysis could identify patients with very aggressive disease, which might benefit from a more intensive treatment.
Databáze:	OpenAIRE
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