Incidence and Survival of Patients With Angioimmunoblastic T-Cell Lymphoma: A Nationwide Population-Based Study Using English Cancer Registration Data.
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Angioimmunoblastic T-cell lymphoma (AITL) is a rare, aggressive subtype of peripheral T-cell lymphoma. Whole population studies of AITL are infrequent and often grouped along with other T-cell lymphomas. We undertook a population-based study using ICD-O3-coded cases of AITL diagnosed 2013-2019 in order to establish AITL epidemiology in England, along with survival outcomes and associated diagnoses. We identified 993 cases, with a median age of 71 years. Overall incidence was 2.57 cases/million person-years (95% CI 2.41-2.74). The incidence increased with age, ranging from 0.24 cases/million person-years at 0-49 years (CI 0.19-0.31) to 11.56 in ≥ 70 years (CI 10.62-12.55), and was lower in females (incidence rate ratio [IRR] 0.74, CI 0.66-0.84). One and 3-year overall survival (OS) were 58% and 37% respectively. 151 patients received auto-SCT, with a 1-year and 3-year OS from transplant of 78.9% and 65.8%. In multivariable analysis, gender (hazard ratio [HR] in females = 0.77, 95% CI 0.66-0.90) and age (HR in ≥ 80 years = 4.41, CI 2.86-6.79) impacted OS. 142 patients developed an associated B-cell lymphoma or Hodgkin lymphoma (14.3%). A diagnosis of autoimmune hemolytic anemia (AIHA) was reported in 56 patients (5.6%), and immune thrombocytopenic purpura (ITP) in 14 (1.4%). Our study defines AITL incidence for the first time in England, confirms OS in an unselected population, and describes rates of autoimmune disease and B-cell lymphomas. Gender and age are the primary characteristics that influence outcomes.