The management of peripheral T-cell lymphoma (PTCL) remains a big challenge. PTCL exists as a collection of subentities, which are all rare. Each subtype described has its own unique pathogenesis, etiological associations and presentation. In general, PTCL is a relatively resistant disorder that exhibits extranodal features, B symptoms and paraneoplastic phenomena. This condition is prone to relapse, with a disappointing overall survival at 5 years of approximately 30%. This review will discuss the differences in the tumor biology of PTCL subentities, their associated targeted therapies, options for first-line treatment and the role of stem cell transplantation in first-line and relapsed settings. The authors then discuss new agents being used in early phase trials in relapsed/refractory disease and discuss the urgent need for collaborative randomized controlled trials in this resistant and biologically aggressive disease group.
Keywords: Bendamustine; Bortezomib; Lenalidomide; Leukemia; Non-Hodgkin's lymphoma; Pralatrexate; T-cell lymphoma.
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