title: Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the international peripheral T-cell lymphoma project.
abstract: OBJECTIVE
  The International Peripheral T-Cell Lymphoma Project was undertaken to better understand the subtypes of T-cell and natural killer (NK) -cell lymphomas.
  
  
  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).
  
  
  CONCLUSIONS
  AITL is a rare clinicopathologic entity characterized by an aggressive course and dismal outcome with current therapies.
authors.affiliation: Università di Modena e Reggio Emilia, Modena, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Federico
authors.forename: Thomas
authors.initials: T
authors.lastname: Rudiger
authors.forename: Monica
authors.initials: M
authors.lastname: Bellei
authors.forename: Bharat N
authors.initials: BN
authors.lastname: Nathwani
authors.forename: Stefano
authors.initials: S
authors.lastname: Luminari
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.forename: Nancy L
authors.initials: NL
authors.lastname: Harris
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: Stefano A
authors.initials: SA
authors.lastname: Pileri
authors.forename: Kerry J
authors.initials: KJ
authors.lastname: Savage
authors.forename: Dennis D
authors.initials: DD
authors.lastname: Weisenburger
authors.forename: James O
authors.initials: JO
authors.lastname: Armitage
authors.forename: Nicholas
authors.initials: N
authors.lastname: Mounier
authors.forename: Julie M
authors.initials: JM
authors.lastname: Vose
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D016130:Immunophenotyping; D016411:Lymphoma, T-Cell, Peripheral / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016896:Treatment Outcome; D055815:Young Adult