Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the International Peripheral T-cell Lymphoma Project
Journal of Clinical Oncology, 08/10/2012
Federico M et al. – AITL is a rare clinicopathologic entity characterized by an aggressive course and dismal outcome with current therapies.
Methods- Angioimmunoblastic T-cell lymphoma (AITL) was diagnosed according to the 2001 WHO criteria by a central review process consisting of panels of expert hematopathologists.
- Clinical, pathologic, immunophenotyping, treatment, and survival data were correlated.
- Of 1,314 patients, 243 (18.5%) were diagnosed with AITL.
- At presentation, generalized lymphadenopathy was noted in 76% of patients, and 89% had stages III to IV disease. Skin rash was observed in 21% of patients.
- Hemolytic anemia and hypergammoglobulinemia occurred in 13% and 30% of patients, respectively.
- Five-year overall and failure-free survivals were 33% and 18%, respectively.
- At presentation, prognostic models were evaluated, including the standard International Prognostic Index, which comprised the following factors: age ? 60 years, stages III to IV disease, lactic dehydrogenase (LDH) > normal, extranodal sites (ENSs) > one, and performance status (PS) ? 2; the Prognostic Index for Peripheral T-Cell Lymphoma, comprising: age ? 60 years, PS ? 2, LDH > normal, and bone marrow involvement; and the alternative Prognostic Index for AITL (PIAI), comprising: age > 60 years, PS ? 2, ENSs > one, B symptoms, and platelet count < 150 × 109/L.
- The simplified PIAI had a low-risk group (zero to one factors), with 5-year survival of 44%, and a high-risk group (two to five factors), with 5-year survival of 24% (P = .0065).
