From the Professor of Medicine, Medical Oncology, Yale Cancer Center, New Haven, CT, Francine.foss{at}yale.edu, 203-737-5312
Authors’ disclosures of potential conflicts of interest are found at the end of this article.
Address reprint requeststo Francine Foss, MD, Yale Cancer Center, Department of Medical Oncology, 333 Cedar St, PO Box 208032 FMP 130, New Haven CT 06520-8032; e-mail: francine.foss{at}yale.edu
Overview: The aggressive T-cell lymphomas are a heterogeneous group of neoplasms, which comprise up to 15% of lymphomas in Europe and North America but are more common in the Far East. With the exception of mycosis fungoides, cutaneous anaplastic large cell lymphoma (ALCL) and large granular lymphocytic leukemia, which have a more indolent course, aggressive T-cell lymphomas are associated with a poor outcome. The International T-cell Lymphoma Project recently reviewed more than 1,300 cases of T-cell lymphomas and demonstrated that, with the exception of the anaplastic lymphoma kinase-positive ALCLs, the other subtypes have similar median 5-year survival rates ranging from 20% to 35%. Although traditional cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-based treatment strategies for T-cell lymphomas have been extrapolated from studies of diffuse large B-cell lymphomas, there is little evidence to suggest that these regimens are optimal in the setting of T-cell histologies. Although most of the data is retrospective, a number of trials have been conducted to explore novel approaches, including prospective trials of first remission consolidation therapy with autologous stem cell transplantation and the addition of agents such as denileukin diftitox or alemtuzumab to chemotherapy. In the setting of localized natural killer (NK)/T-cell lymphomas, treatment often includes involved field radiotherapy, which has been shown to significantly improve outcomes. A number of promising agents that demonstrate activity in relapsed and refractory T-cell lymphomas have recently been developed and are being explored in combination studies.
