Outcomes and prognostic factors in angioimmunoblastic T-cell lymphoma: final report from the international T-cell Project

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a unique subtype of peripheral T-cell lymphoma (PTCL) with distinct clinicopathologic features and poor prognosis. We performed a subset analysis of 282 patients with AITL enrolled between 2006 and 2018 in the international prospective T-cell Project (NCT01142674). The primary and secondary end points were 5-year overall survival (OS) and progression-free survival (PFS), respectively. We analyzed the prognostic impact of clinical covariates and progression of disease within 24 months (POD24) and developed a novel prognostic score. The median age was 64 years, and 90% of patients had advanced-stage disease. Eighty-one percent received anthracycline-based regimens, and 13% underwent consolidative autologous stem cell transplant (ASCT) in first complete remission (CR1). Five-year OS and PFS estimates were 44% and 32%, respectively, with improved outcomes for patients who underwent ASCT in CR1. In multivariate analysis, age ≥60 years, Eastern Cooperative Oncology Group performance status >2, elevated C-reactive protein, and elevated β2 microglobulin were associated with inferior outcomes. A novel prognostic score (AITL score) combining these factors defined low-, intermediate-, and high-risk subgroups with 5-year OS estimates of 63%, 54%, and 21%, respectively, with greater discriminant power than established prognostic indices. Finally, POD24 was a powerful prognostic factor with 5-year OS of 63% for patients without POD24 compared with only 6% for patients with POD24 (P < .0001). These data will require validation in a prospective cohort of homogeneously treated patients. Optimal treatment of AITL continues to be an unmet need, and novel therapeutic approaches are required.

Graphical abstract

Figure 1.

OS and PFS. Kaplan-Meier curves show OS (A) and PFS (B) estimates for the entire study cohort. The 3- and 5-year OS and PFS estimates are listed with 95% CIs. The number of patients under follow-up is listed under the x-axis.

Figure 2.

OS and PFS stratified by transplant status in CR1. Kaplan-Meier curves show OS (A) and PFS (B) estimates for patients who underwent autologous stem cell transplantation in first complete remission (red line) vs transplant-eligible patients (age ≤65) who did not undergo transplantation (blue line). The number of patients under follow-up is listed under the x-axis.

Figure 3.

OS and PFS stratified by POD24. Kaplan-Meier estimates of OS (A) and PFS (B) are shown for patients experiencing POD24 (blue line) or without POD24 (red line). The number of patients under follow-up is listed under the x-axis. Outcomes were superior for patients without POD24 (log-rank value of P < .0001 for both comparisons).