[Peripheral T-cell lymphoma: diagnosis and treatment].
Peripheral T/NK-cell lymphomas (PTCL) comprise a heterogeneous group of rare diseases accounting for approximately 10-15% of all non-Hodgkin"s lymphomas. Compared to B-cell lymphomas, PTCL more frequently involve extranodal sites and have a worse prognosis. Because of their usually indolent course, primary cutaneous T/NK-cell lymphomas should be distinguished from the other PTCL. Staging of PTCL is done according to the Ann Arbor staging system. The International Prognostic Index, established for aggressive B-cell lymphomas, has also proved relevant for PTCL. Standard therapy for PTCL has not been defined, yet. First line anthracycline-based chemotherapy brings about long-term remissions in 15 to 42% of patients. Apart from (ALK-positive) anaplastic large cell lymphoma it thus gives poorer results than those obtained in patients with aggressive B-cell lymphomas. Data for high-dosage therapy with autologous stem cell transplantation (autoSCT) for relapsing and refractory PTCL are similar to those reported for aggressive B-cell lymphomas. Thus this treatment seems to constitute a sensible salvage strategy. Allogeneic stem cell transplantation following reduced conditioning regimens has also given promising results in patients with relapse. However, the impact of high-dosage strategies and the implementation of newer agents, such as alemtuzumab, in first-line treatment are still uncertain. Hence patients with PTCL should be treated within clinical trials.
Medizinische Klinik und Poliklinik II, Julius-Maximilians-Universität. p.reimer@medizin.uni-wuerzburg.de
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D006801:Humans; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000532:radiotherapy / Q000628:therapy*; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D014184:Transplantation, Homologous