Peripheral (post-thymic) T-cell lymphoma consists of a wide spectrum of disorders with marked differences in biology and behavior. Proper classification is pivotal for evaluating treatment results, and most studies performed a decade ago lump together different disease entities and cannot be interpreted. With improved use of immunophenotyping and molecular methods for these disorders, their exact nature is better defined in the Revised European-American Lymphoma and subsequent World Health Organization (WHO) classifications. The WHO classification of post- thymic T/natural killer (NK)-cell lymphoma consists of 15 entities, including about 30% that are unclassified cases. A wide range in incidence exists between different populations, but it is likely to be lower than previously estimated. Certain entities, like nasal/nasal-type T/NK-cell lymphoma and human T-cell leukemia/lymphoma virus 1, are much more prevalent in certain racial groups and show exquisite viral association. In these entities as a group, prognosis and treatment seem inferior to those of their B-cell counterparts, but treatment must be tailored to the exact pathologic diagnosis and prognostic index. Aggressive combination chemotherapy appears to be curative for certain entities (eg, anaplastic lymphoma kinase-positive), whereas purine analogues may be useful for low-grade entities. The role of autologous and allogeneic stem cell transplantation is still poorly defined. Specific antibody-based therapy is also on the horizon.

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