FRIDAY, March 25, 2022 (HealthDay News) — Adding the proteasome inhibitor bortezomib to a chemotherapy regimen improves survival in children with T-cell lymphoblastic lymphoma (T-LL), according to a study published online March 10 in the Journal of Clinical Oncology.

David T. Teachey, M.D., from the Children’s Hospital of Philadelphia, and colleagues randomly assigned 824 children and young adults with T-cell acute lymphoblastic leukemia (T-ALL) and T-LL to modified augmented Berlin-Frankfurt-Münster (aBFM) chemotherapy regimen with/without bortezomib (Arms A and B, respectively) in the Children’s Oncology Group phase 3 clinical trial AALL1231. In an attempt to eliminate use of prophylactic cranial radiation (CRT) in most patients, modifications were made to the aBFM backbone used in the predecessor trial AALL0434.

The researchers found that for Arms A and B, the four-year event-free survival (EFS) was 80.1 ± 2.3 versus 83.8 ± 2.1 percent and OS was 85.7 ± 2.0 versus 88.3 ± 1.8 percent, respectively. Improved EFS and OS was seen with bortezomib for T-LL patients: four-year EFS: 76.5 ± 5.1 versus 86.4 ± 4.0 percent; four-year OS: 78.3 ± 4.9 versus 89.5 ± 3.6 percent. There was no excess toxicity observed with bortezomib. Overall, 90.8 percent of T-ALL patients received CRT on AALL0434, compared with 9.5 percent of patients in AALL1231 who were scheduled to receive CRT. No significant differences in EFS or OS were seen for AALL0434 patients who received CRT and AALL1231 patients who did not receive CRT.

“The data show that most patients with T-ALL no longer need cranial radiation for cure and also suggest bortezomib should be considered as part of the new standard of care for newly diagnosed patients with T-cell lymphoblastic lymphoma,” Teachey said in a statement.

Several authors disclosed financial ties to pharmaceutical companies, including Amgen, the manufacturer of bortezomib.

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