title: How I manage peripheral T-cell lymphoma, not otherwise specified and angioimmunoblastic T-cell lymphoma: current practice and a glimpse into the future.
abstract: Peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS) and angioimmunoblastic T-cell lymphoma (AITL) are the most frequent of more than 20 mature PTCL entities featuring a broad spectrum of morphological, immunophenotypic, molecular and clinical characteristics. Unfortunately, recent progress in understanding the (epi)genetic background of PTCL has not been met with similar advances in treatment. Thus, CHO(E)P [cyclophosphamide, doxorubicin, vincristine, and prednisone (plus etoposide)] remains standard first-line therapy. Patients without comorbidities achieving complete or partial remission proceed to autologous stem cell transplantation. With this approach about 50% of patients survive long-term. Patients relapsing after or progressing during first-line therapy have a dismal prognosis. They receive salvage gemcitabine-therapy followed by allogeneic transplantation whenever possible. After allografting, approximately half of the patients survive long-term; any other treatment is palliative. New drugs investigated in phase II studies achieved response rates between 10% and 30%; long-term remissions are the exception to the rule. While most new drugs are not licensed and not readily available, a plethora of other innovative drugs targeting (epi-)genetic abnormalities are in early development. These, together with combinations of new and old drugs, will hopefully increase response to first-line therapy, bridge more patients to transplantation, and finally improve prognosis for all patients with PTCL.
authors.affiliation: Department of Haematology, Oncology and Stem Cell Transplantation, Asklepios Hospital St. Georg, Hamburg, Germany.
authors.forename: Norbert
authors.initials: N
authors.lastname: Schmitz
authors.affiliation: Institute of Pathology, University Hospital Lausanne and University of Lausanne, Switzerland.
authors.forename: Laurence
authors.initials: L
authors.lastname: de Leval
mesh_terms: D003131:Combined Modality Therapy / Q000009:adverse effects / Q000379:methods; D003937:Diagnosis, Differential; D019468:Disease Management; D055808:Drug Discovery; D019008:Drug Resistance, Neoplasm; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000175:diagnosis / Q000628:therapy; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D058990:Molecular Targeted Therapy; D009367:Neoplasm Staging; D010818:Practice Patterns, Physicians'; D011379:Prognosis; D012008:Recurrence; D059039:Standard of Care; D016896:Treatment Outcome
keywords: Peripheral T-cell lymphoma; angioimmunoblastic T-cell lymphoma; pathology; personalized; stem cell transplantation; treatment