TY - JOUR
T1 - Peripheral T-cell lymphoma, not otherwise specified
T2 - A report of 340 cases from the International Peripheral T-cell Lymphoma Project
AU - Weisenburger, Dennis D.
AU - Savage, Kerry J.
AU - Harris, Nancy Lee
AU - Gascoyne, Randy D.
AU - Jaffe, Elaine S.
AU - MacLennan, Kenneth A.
AU - Rüdiger, Thomas
AU - Pileri, Stefano
AU - Nakamura, Shigeo
AU - Nathwani, Bharat
AU - Campo, Elias
AU - Berger, Francoise
AU - Coiffier, Bertrand
AU - Kim, Won Seog
AU - Holte, Harald
AU - Federico, Massimo
AU - Au, Wing Y.
AU - Tobinai, Kensei
AU - Armitage, James O.
AU - Vose, Julie M.
PY - 2011/3/24
Y1 - 2011/3/24
N2 - 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 × 109/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.
AB - 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 × 109/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.
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U2 - 10.1182/blood-2010-09-310342
DO - 10.1182/blood-2010-09-310342
M3 - Article
C2 - 21270441
AN - SCOPUS:79953124083
VL - 117
SP - 3402
EP - 3408
JO - Blood
JF - Blood
SN - 0006-4971
IS - 12
ER -