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, International Peripheral T-cell Lymphoma Project, Kerry Savage, Joseph Connors, Mukesh Chhanabhai, Randy Gascoyne, Wyndham Wilson, Elaine Jaffe, James Armitage, Julie Vose, Dennis Weisenburger, James Anderson, Fred Ullrich, Martin Bast, Ephraim Hochberg, Agata Smogorzewska, Nancy Harris, Alexandra Levine, Bharat Nathwani, Thomas Miller, Lisa Rimsza, Emili Montserrat, Armando Lopez-Guillermo, Elias Campo, Marta Cuadros, Javier Alvarez Ferreira, Beatriz Martinez Delgado, Harald Holte, Jan Delabie, Thomas Rüdiger, Konrad Müller-Hermelink, Peter Reimer, Patrick Adam, Martin Wilhelm, Norbert Schmitz, Christoph Nerl, Andrew Lister, Andrew Norton, Kenneth A MacLennan, Pier Luigi Zinzani, Stefano Pileri, Massimo Federico, Monica Bellei, Bertrand Coiffier, Francoise Berger, Intragumtornchai Tanin, Pongsak Wannakrairot, Wing Au, Raymond Liang, Florence Loong, Sandeep Rajan, Ivy Sng, Kensei Tobinai, Yoshihiro Matsuno, Yasuo Morishima, Shigeo Nakamura, Masao Seto, Mitsune Tanimoto, Tadashi Yoshino, Junji Suzumiya, Koichi Ohshima, Won-Seog Kim, Young-Hyeh Ko, 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, International Peripheral T-cell Lymphoma Project, Kerry Savage, Joseph Connors, Mukesh Chhanabhai, Randy Gascoyne, Wyndham Wilson, Elaine Jaffe, James Armitage, Julie Vose, Dennis Weisenburger, James Anderson, Fred Ullrich, Martin Bast, Ephraim Hochberg, Agata Smogorzewska, Nancy Harris, Alexandra Levine, Bharat Nathwani, Thomas Miller, Lisa Rimsza, Emili Montserrat, Armando Lopez-Guillermo, Elias Campo, Marta Cuadros, Javier Alvarez Ferreira, Beatriz Martinez Delgado, Harald Holte, Jan Delabie, Thomas Rüdiger, Konrad Müller-Hermelink, Peter Reimer, Patrick Adam, Martin Wilhelm, Norbert Schmitz, Christoph Nerl, Andrew Lister, Andrew Norton, Kenneth A MacLennan, Pier Luigi Zinzani, Stefano Pileri, Massimo Federico, Monica Bellei, Bertrand Coiffier, Francoise Berger, Intragumtornchai Tanin, Pongsak Wannakrairot, Wing Au, Raymond Liang, Florence Loong, Sandeep Rajan, Ivy Sng, Kensei Tobinai, Yoshihiro Matsuno, Yasuo Morishima, Shigeo Nakamura, Masao Seto, Mitsune Tanimoto, Tadashi Yoshino, Junji Suzumiya, Koichi Ohshima, Won-Seog Kim, Young-Hyeh Ko
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.
Source: PubMed