| Autor: |
Newman, Alexander M., Zaka, Masood, Zhou, Peixun, Blain, Alex E., Erhorn, Amy, Barnard, Amy, Crossland, Rachel E., Wilkinson, Sarah, Enshaei, Amir, De Zordi, Julian, Harding, Fiona, Taj, Mary, Wood, Katrina M., Televantou, Despina, Turner, Suzanne D., Burke, G. A. Amos, Harrison, Christine J., Bomken, Simon, Bacon, Chris M., Rand, Vikki |
| Zdroj: |
Leukemia; March 2022, Vol. 36 Issue: 3 p781-789, 9p |
| Abstrakt: |
Children with B-cell non-Hodgkin lymphoma (B-NHL) have an excellent chance of survival, however, current clinical risk stratification places as many as half of patients in a high-risk group receiving very intensive chemo-immunotherapy. TP53alterations are associated with adverse outcome in many malignancies; however, whilst common in paediatric B-NHL, their utility as a risk classifier is unknown. We evaluated the clinical significance of TP53abnormalities (mutations, deletion and/or copy number neutral loss of heterozygosity) in a large UK paediatric B-NHL cohort and determined their impact on survival. TP53abnormalities were present in 54.7% of cases and were independently associated with a significantly inferior survival compared to those without a TP53abnormality (PFS 70.0% vs 100%, p< 0.001, OS 78.0% vs 100%, p= 0.002). Moreover, amongst patients clinically defined as high-risk (stage III with high LDH or stage IV), those without a TP53abnormality have superior survival compared to those with TP53abnormalities (PFS 100% vs 55.6%, p= 0.005, OS 100% vs 66.7%, p= 0.019). Biallelic TP53abnormalities were either maintained from the presentation or acquired at progression in all paired diagnosis/progression Burkitt lymphoma cases. TP53abnormalities thus define clinical risk groups within paediatric B-NHL and offer a novel molecular risk stratifier, allowing more personalised treatment protocols. |
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