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. In this...

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. 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).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.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).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

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