The aggressive peripheral T‐cell lymphomas: 2013

BackgroundT‐cell lymphomas make up approximately 10–15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia. DiagnosisThe diagnosis of aggressive peripheral T‐cell lymphoma (PTCL) is usually made using the WHO classification. The ability of hematopathologists to reproducibly diagnosis aggressive PTCL is lower than for aggressive B‐cell lymphomas, with a range of 72–97% for the aggressive PTCLs. Risk StratificationPatients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, PET scans appear to be useful as they are in aggressive B‐cell lymphomas. The most commonly used prognostic index is the International Prognostic Index. The specific subtype of aggressive PTCL is an important risk factor, with the best survival seen in anaplastic large cell lymphoma—particularly young patients with the anaplastic lymphoma kinase positive subtype. Risk Adapted TherapyAnaplastic large cell lymphoma is the only subgroup to have a good response to a CHOP‐like regimen. Angioimmunoblastic T‐cell lymphoma has a prolonged disease‐free survival in only ∼20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL‐NOS (not otherwise specified) is not one disease. Anthracycline containing regimens have disappointing results and a new approach is needed. NK/T‐cell lymphoma localized to the nose and nasal sinuses see...
Source: American Journal of Hematology - Category: Hematology Authors: Tags: Annual Clinical Updates in Hematological Malignancies: A Continuing Medical Education Series Source Type: research