Peripheral T-cell lymphoma, not otherwise specified: A report of 340 cases from the International Peripheral T-cell Lymphoma Project

Dennis D. Weisenburger, Kerry J. Savage, Nancy Lee Harris, Randy D. Gascoyne, Elaine S. Jaffe, Kenneth A. MacLennan, Thomas Rüdiger, Stefano Pileri, Shigeo Nakamura, Bharat Nathwani, Elias Campo, Francoise Berger, Bertrand Coiffier, Won Seog Kim, Harald Holte, Massimo Federico, Wing Y. Au, Kensei Tobinai, James O. Armitage, Julie M. Vose

Research output: Contribution to journalArticle

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 × 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.

Original languageEnglish
Pages (from-to)3402-3408
Number of pages7
JournalBlood
Volume117
Issue number12
DOIs
Publication statusPublished - Mar 24 2011

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

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    Weisenburger, D. D., Savage, K. J., Harris, N. L., Gascoyne, R. D., Jaffe, E. S., MacLennan, K. A., Rüdiger, T., Pileri, S., Nakamura, S., Nathwani, B., Campo, E., Berger, F., Coiffier, B., Kim, W. S., Holte, H., Federico, M., Au, W. Y., Tobinai, K., Armitage, J. O., & Vose, J. M. (2011). Peripheral T-cell lymphoma, not otherwise specified: A report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood, 117(12), 3402-3408. https://doi.org/10.1182/blood-2010-09-310342