Abstract

clinical outcome compared with B-cell lymphomas, with the exception of ALK-positive anaplastic large-cell lymphoma. Long-term survival at 5 years remains at 10%-30% for most histologies with current treatment strategies, as compared with 55% for diffuse large B-cell lymphoma.4 Advanced disease stage, high International Prognostic Index at presentation, and inherent chemoresistance contribute to this dismal outcome.5,6 Most patients are treated with CHOP (cyclophosphamide/ doxorubicin/vincristine/prednisone)/CHOP-like regimens as the first line of therapy adopted from the management of aggressive B-cell lymphomas, but the outcome remains poor.7 Relapsed and chemorefractory disease remains a significant clinical dilemma in the care of these patients. The rarity and heterogeneity of these diseases makes it difficult to do well-conducted clinical trials; hence, there are no well-defined standards for the treatment of these diseases either in the first-line setting or for relapsed disease. There is a need to develop new targeted treatment options for these patients based on an understanding of the pathogenesis of these lymphomas. Emerging new therapeutic agents for the treatment of relapsed/refractory PTCL are described herein.

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