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Background <p>Peripheral T‑cell lymphomas (PTCL) encompass a&#xa0;diverse group of rare and aggressive mature T‑cell neoplasms, classified according to WHO HAEM5 or ICC 2022. The management of PTCL remains challenging due to the disease’s biological heterogeneity, low incidence, and the scarcity of robust data from large clinical trials, thus limiting the development of evidence-based treatment strategies.</p> Objective <p>This article outlines current advances in treating common forms of nodal PTCL from first-line to relapse therapy, highlights the importance of novel treatment strategies, and discusses future approaches.</p> Materials and methods <p>The content presented is grounded in clinical study findings and aligns with the latest guidelines.</p> Results <p>Accurate pathological classification is essential for treatment planning, enabling the consideration of subtype-specific therapeutic approaches when necessary. For the main types of nodal T‑cell lymphomas, CHOP-based chemotherapy remains the standard first-line treatment, with BV-CHP (replacing vincristine with brentuximab vedotin) recommended for patients with anaplastic large cell lymphoma (ALCL). Based on phase&#xa0;II studies, treatment intensification with etoposide (CHOEP) and consolidation with high-dose therapy followed by autologous stem cell transplantation (SCT) are viable options for eligible patients. In cases of refractory or relapsed disease, platinum- or gemcitabine-based regimens, as well as brentuximab vedotin for ALCL, can provide disease control. Allogeneic SCT is available for suitable patients with curative intent. Ongoing research is focused on integrating molecularly and epigenetically targeted therapies into personalized treatment concepts as well as advancing allogeneic SCT and cellular immunotherapy.</p> Conclusion <p>Patients with PTCL should, if possible, be treated within the framework of clinical studies, with increasing emphasis on entity-specific and patient-tailored therapies.</p>

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Therapie der nodalen peripheren T-Zell-Lymphome

  • 期刊论文

  • 发表日期：

    2025年4月1日

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  • Till Braun,
  • Gerald Wulf

摘要

Background

Peripheral T‑cell lymphomas (PTCL) encompass a diverse group of rare and aggressive mature T‑cell neoplasms, classified according to WHO HAEM5 or ICC 2022. The management of PTCL remains challenging due to the disease’s biological heterogeneity, low incidence, and the scarcity of robust data from large clinical trials, thus limiting the development of evidence-based treatment strategies.

Objective

This article outlines current advances in treating common forms of nodal PTCL from first-line to relapse therapy, highlights the importance of novel treatment strategies, and discusses future approaches.

Materials and methods

The content presented is grounded in clinical study findings and aligns with the latest guidelines.

Results

Accurate pathological classification is essential for treatment planning, enabling the consideration of subtype-specific therapeutic approaches when necessary. For the main types of nodal T‑cell lymphomas, CHOP-based chemotherapy remains the standard first-line treatment, with BV-CHP (replacing vincristine with brentuximab vedotin) recommended for patients with anaplastic large cell lymphoma (ALCL). Based on phase II studies, treatment intensification with etoposide (CHOEP) and consolidation with high-dose therapy followed by autologous stem cell transplantation (SCT) are viable options for eligible patients. In cases of refractory or relapsed disease, platinum- or gemcitabine-based regimens, as well as brentuximab vedotin for ALCL, can provide disease control. Allogeneic SCT is available for suitable patients with curative intent. Ongoing research is focused on integrating molecularly and epigenetically targeted therapies into personalized treatment concepts as well as advancing allogeneic SCT and cellular immunotherapy.

Conclusion

Patients with PTCL should, if possible, be treated within the framework of clinical studies, with increasing emphasis on entity-specific and patient-tailored therapies.