Overview
Peripheral T-cell lymphoma (PTCL) represents a heterogeneous group of non-Hodgkin lymphomas originating from mature T-cells, accounting for approximately 10%-15% of all lymphomas. Treatment outcomes vary significantly across subtypes, with current standards often falling short of optimal efficacy 23.Diagnosis
Comprehensive histopathological examination including immunohistochemistry is essential for accurate diagnosis 3.
Flow cytometry and molecular studies may aid in subtype classification 3.
Clinical staging using the Ann Arbor staging system is crucial for treatment planning 34.Management
First-line treatment: CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) remains the standard, though outcomes are often suboptimal 23.
Adjunctive therapies:
- Addition of brentuximab vedotin improves outcomes in anaplastic large cell lymphoma 2.
- Autologous stem cell transplantation in first complete remission, except for ALK+ anaplastic large cell lymphoma, shows promise 2.
- Allogeneic hematopoietic cell transplantation (HCT) is considered for refractory or relapsed cases, leveraging curative potential 1.
Specific drug approvals: Beleodaq (histone deacetylase inhibitor) approved for relapsed or refractory PTCL 5.Special Populations
Elderly patients: Often present with advanced stage disease and comorbidities; anthracycline-based (CHOP, CHOEP) and non-anthracycline-based (IMEP, CVP) chemotherapies show comparable response rates 4.
Outcomes: Median 5-year overall survival (OS) around 45.9%, with significant variability based on comorbidity and stage 4.Key Recommendations
Consider allogeneic HCT for eligible patients with refractory or relapsed PTCL to harness its curative potential (Evidence: Expert opinion) 1.
Utilize CHOP as the standard first-line chemotherapy regimen, while monitoring for suboptimal outcomes and exploring alternative treatments 23.
Evaluate autologous stem cell transplantation in first complete remission, excluding ALK+ anaplastic large cell lymphoma patients, to enhance disease control (Evidence: Moderate) 2.
Consider brentuximab vedotin for patients with anaplastic large cell lymphoma to improve treatment outcomes (Evidence: Strong) 2.
Monitor and manage comorbidities carefully in elderly patients, as they significantly impact survival rates despite comparable response rates to different chemotherapy regimens (Evidence: Moderate) 4.References
1 Damaj G, Bazarbachi A, Berning P, Cottereau AS, Fox CP, Kyriakou C et al.. Allogeneic haematopoietic cell transplantation in peripheral T-cell lymphoma: recommendations from the EBMT Practice Harmonisation and Guidelines Committee. The Lancet. Haematology 2025. link00073-0)
2 Milunović V, Smoljanović IM, Patekar MB, Zatezalo V, Kursar M, Radić-Krišto D et al.. First-Line Therapy for Nodal T-cell Non-Hodgkin Lymphomas: an Unmet Need in Hematology. Current oncology reports 2023. link
3 Kayıkçı Ö, Mehtap Ö, Sarı İ, Demirkan F, Beyan C, Çetin G et al.. Real-Life Experience Regarding Clinical Characteristics and Treatment Outcome in Non-Cutaneous Peripheral T-Cell Lymphomas: A Multicenter Study of the Turkish Hematology Research and Education Group (ThREG). Turkish journal of haematology : official journal of Turkish Society of Haematology 2022. link
4 Jo JC, Choi Y, Shin HJ, Yhim SN, Lee HS, Lee WS et al.. Peripheral T cell lymphomas in elderly patients: a retrospective analysis from the Hematology Association of South East Korea (HASEK). Annals of hematology 2016. link
5 . Beleodaq approved for rare lymphomas. Cancer discovery 2014. link