Clinicopathologic characteristics of angioimmunoblastic t-cell lymphoma: Analysis of the international peripheral t-cell lymphoma project

Massimo Federico, Thomas Rudiger, Monica Bellei, Bharat N. Nathwani, Stefano Luminari, Bertrand Coiffier, Nancy L. Harris, Elaine S. Jaffe, Stefano A. Pileri, Kerry J. Savage, Dennis D. Weisenburger, James O. Armitage, Nicholas Mounie, Julie M. Vose

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274 Scopus citations


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

Original languageEnglish (US)
Pages (from-to)240-246
Number of pages7
JournalJournal of Clinical Oncology
Issue number2
StatePublished - Jan 10 2013

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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