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

• Published in: Blood 2021-07, Vol.138 (3), p.213-220
• Main Authors: Advani, Ranjana H., Skrypets, Tetiana, Civallero, Monica, Spinner, Michael A., Manni, Martina, Kim, Won Seog, Shustov, Andrei R., Horwitz, Steven M., Hitz, Felicitas, Cabrera, Maria Elena, Dlouhy, Ivan, Vassallo, José, Pileri, Stefano A., Inghirami, Giorgio, Montoto, Silvia, Vitolo, Umberto, Radford, John, Vose, Julie M., Federico, Massimo
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Clinical Trials and Observations; Disease Progression; Female; Humans; Immunoblastic Lymphadenopathy - diagnosis; Immunoblastic Lymphadenopathy - drug therapy; Immunoblastic Lymphadenopathy - therapy; Lymphoma, T-Cell, Peripheral - diagnosis; Lymphoma, T-Cell, Peripheral - drug therapy; Lymphoma, T-Cell, Peripheral - therapy; Male; Middle Aged; Prognosis; Stem Cell Transplantation; T-Lymphocytes - pathology; Transplantation, Autologous; Treatment Outcome; Young Adult
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.2020010387

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. •Contemporary treatment approaches for AITL remain suboptimal.•A novel prognostic AITL score defined low-, intermediate-, and high-risk subgroups with differing outcomes. [Display omitted]