Photo Credit: Nemes Laszlo

The following is a summary of “Clinical significance of NOTCH1 and FBXW7 alterations in adult T-cell leukemia/lymphoma,” published in the November 2024 issue of Hematology by Sakamoto et al. 

Researchers conducted a retrospective study to examine the clinical significance of NOTCH1 and FBXW7 alterations in adult T-cell leukemia/lymphoma (ATLL). 

They analyzed 257 patients, identifying NOTCH1 alterations in 37 (14.4%), including 33 single nucleotide variants/insertions-deletions in the PEST domain and 7 in the heterodimerization or LIN-12/Notch repeats domains. FBXW7 alterations were observed in 9 (3.5%) patients. 

The results showed that NOTCH1 alterations were independently associated with worse overall survival (OS) in patients without allogeneic hematopoietic stem cell transplantation (HSCT). For 27 patients with NOTCH1 alterations, median survival was 0.5 years (95% CI 0.4–0.5), compared to 1.8 years (95% CI 1.3–2.2) for 170 without. In patients receiving mogamulizumab, median survival was 0.4 years (95% CI 0.3–0.5) for 12 with NOTCH1 alterations versus 1.4 years (95% CI 0.9–2.0) for 87 without. No significant impact was found in patients who received allogeneic-HSCT. 

Mogamulizumab-containing treatment was unable to overcome treatment refractoriness in ATLL with NOTCH1 alterations. Patients with NOTCH1 alterations are recommended for allogeneic-HSCT. 

Source: link.springer.com/article/10.1007/s12185-024-03880-3