Angioimmunoblastic T-Cell Lymphoma: Report on 282 Cases from the Prospective International T-Cell Lymphoma Project

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare and unique subtype of peripheral T-cell lymphoma (PTCL) with distinct clinicopathologic features. Clinical presentation is varied with an aggressive course and dismal outcomes. Curative treatment modalities in AITLs are still an unmet need. Aims: In this study, we aimed to advance our understanding of clinical characteristics, prognostic factors, treatment strategies in patients with AITL in the international prospective T-Cell Project (TCP). Patients and methods: We did a sub-analysis of 282 patients with AITL out of 1,553 cases enrolled between 2006 and 2018 in the TCP, a global prospective registry of patients with PTCL involving 74 institutions in 13 countries in Europe, North/South America, and Asia. Eligible patients were >18 years old with baseline clinical data necessary for disease staging, treatment type, and follow-up for at least 5 years. The primary and secondary endpoints were 5-year OS and PFS. Additionally, we analyzed prognostic factors and POD24. The TCP is registered on ClinicalTrials.gov , NCT01142674 . Results: The median age at diagnosis was 64 years (range 22–88) and 63% of patients were >60 years old. The advanced stage had 90% of patients and 60% were males. According to the IPI, PIT, and PIAI, the majority of cases were in the high-risk groups. Anthracyclinecontaining chemotherapy was received by 81% of patients, and 27 (12.5%) underwent HDT/ASCT as consolidation. Five-year OS and PFS were 44% and 32%, respectively. CR was achieved in 106 patients. ASCT was associated with superior OS (89% vs 52%, p = 0.05) and PFS (79% vs 31, p = 0.02). In multivariate analysis, older age (p=0.003), ECOG PS >2 (p=0.0001), CRP>ULN (p=0.003) and Beta2 microglobulin >ULN (p=0.002) showed an independent prognostic value on PFS. Finally, POD24 was a powerful predictor of outcome: the 5-year PFS for patients with or without POD24 was 2% and 48%, respectively (p=0.0001). Conclusions: Our data confirmed the poor outcome of AITL, mostly in patients exhibiting POD24, and the promising efficacy of ASCT in CR1. Moreover, the collected data gave a better understanding of the need for more effective therapies and the importance to continue prospective studies in a real-world population.