title: Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project.
abstract: The International Peripheral T-cell Lymphoma Project is a collaborative effort to better understand peripheral T-cell lymphoma (PTCL). A total of 22 institutions submitted clinical and pathologic material on 1314 cases. One objective was to analyze the clinical and pathologic features of 340 cases of PTCL, not otherwise specified. The median age of the patients was 60 years, and the majority (69%) presented with advanced stage disease. Most patients (87%) presented with nodal disease, but extranodal disease was present in 62%. The 5-year overall survival was 32%, and the 5-year failure-free survival was only 20%. The majority of patients (80%) were treated with combination chemotherapy that included an anthracycline, but there was no survival advantage. The International Prognostic Index (IPI) was predictive of both overall survival and failure-free survival (P < .001). Multivariate analysis of clinical and pathologic prognostic factors, respectively, when controlling for the IPI, identified bulky disease (≥ 10 cm), thrombocytopenia (< 150 × 10(9)/L), and a high number of transformed tumor cells (> 70%) as adverse predictors of survival, but only the latter was significant in final analysis. Thus, the IPI and a single pathologic feature could be used to stratify patients with PTCL-not otherwise specified for novel and risk-adapted therapies.
authors.affiliation: Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198-3135, USA. dweisenb@unmc.edu
authors.forename: Dennis D
authors.initials: DD
authors.lastname: Weisenburger
authors.forename: Kerry J
authors.initials: KJ
authors.lastname: Savage
authors.forename: Nancy Lee
authors.initials: NL
authors.lastname: Harris
authors.forename: Randy D
authors.initials: RD
authors.lastname: Gascoyne
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: Kenneth A
authors.initials: KA
authors.lastname: MacLennan
authors.forename: Thomas
authors.initials: T
authors.lastname: Rüdiger
authors.forename: Stefano
authors.initials: S
authors.lastname: Pileri
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.forename: Bharat
authors.initials: B
authors.lastname: Nathwani
authors.forename: Elias
authors.initials: E
authors.lastname: Campo
authors.forename: Francoise
authors.initials: F
authors.lastname: Berger
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.forename: Won-Seog
authors.initials: WS
authors.lastname: Kim
authors.forename: Harald
authors.initials: H
authors.lastname: Holte
authors.forename: Massimo
authors.initials: M
authors.lastname: Federico
authors.forename: Wing Y
authors.initials: WY
authors.lastname: Au
authors.forename: Kensei
authors.initials: K
authors.lastname: Tobinai
authors.forename: James O
authors.initials: JO
authors.lastname: Armitage
authors.forename: Julie M
authors.initials: JM
authors.lastname: Vose
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007391:International Cooperation; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult