T-cell lymphomas are a type of non-Hodgkin lymphoma that arise from specific immune system cells termed T lymphocytes. This relatively rare lymphoma is generally classified as either cutaneous T-cell lymphoma (CTCL) or peripheral T-cell lymphoma (PTCL). While CTCL initially manifests as skin lesions before metastasizing to other organs, PTCL may occur both systemically or cutaneously. The most common forms of PTCL are the nodal subtypes, including PTCL not otherwise specified, angioimmunoblastic T-cell lymphoma, and anaplastic large cell lymphoma (ALCL). Unfortunately, most cases of nodal PTCL are aggressive and respond poorly to available treatments. An increased understanding of the molecular and immunologic characteristics of PTCL has promoted the investigation of several agents for both the frontline and relapsed/refractory setting. One of these, pralatrexate, received US Food and Drug Administration approval in the fall of 2009. This represented a major advancement in the treatment of PTCL, as this is the first and only drug to receive approval for relapsed/refractory PTCL. Together, the rare and aggressive nature of this disease, the increasing number of clinical trials under way to test new treatment strategies, and the many special issues to be considered when managing PTCL patients indicate that patient care providers should be educated on the most current understanding of this disease.

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