Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the international peripheral T-cell lymphoma project

Abstract

Purpose: The International Peripheral T-Cell Lymphoma Project was undertaken to better understand the subtypes of T-cell and natural killer (NK) -cell lymphomas.

Patients and 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.

Results: 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 × 10(9)/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).

Conclusion: AITL is a rare clinicopathologic entity characterized by an aggressive course and dismal outcome with current therapies.

Fig 1.

Overall and failure-free survival for 243 patients with angioimmunoblastic T-cell lymphoma.

Fig 2.

Overall survival (OS) for patients with angioimmunoblastic T-cell lymphoma (AITL) using the (A) International Prognostic Index, (B) Prognostic Index for Peripheral T-Cell Lymphoma, Unspecified (PIT), and (C) Prognostic Index for AITL (PIAI); (D) OS for GELA (Groupe d'Etude des Lymphomes de l'Adulte) validation cohort using the PIAI.

Fig 3.

Failure-free survival (FFS) for patients with angioimmunoblastic T-cell lymphoma (AITL) using the (A) International Prognostic Index, (B) Prognostic Index for Peripheral T-Cell Lymphoma, Unspecified (PIT), and (C) Prognostic Index for AITL (PIAI); (D) FFS for GELA (Groupe d'Etude des Lymphomes de l'Adulte) validation cohort using the PIAI.