The aggressive peripheral T-cell lymphomas: 2013

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Peripheral T-cell lymphomas (PTCL) constitute 10-15% of lymphoid malignancies.
• PTCL incidence shows geographic variation, notably higher in Asia.
• Accurate diagnosis of aggressive PTCL is challenging for hematopathologists compared to aggressive B-cell lymphomas.

Purpose of the Study:

• To review the diagnostic criteria, staging, prognostic factors, and risk-adapted therapies for aggressive peripheral T-cell lymphomas.
• To highlight the heterogeneity of PTCL and the need for subtype-specific treatment approaches.

Main Methods:

• Utilizes the World Health Organization (WHO) classification for PTCL diagnosis.
• Employs the Ann Arbor Classification and International Prognostic Index (IPI) for staging and prognostication.
• Reviews treatment responses for various PTCL subtypes, including CHOP-like regimens, autotransplantation, and radiotherapy.

Main Results:

• Anaplastic large cell lymphoma (ALCL), especially the anaplastic lymphoma kinase (ALK)-positive subtype, shows better survival.
• ALCL is the only subgroup responding well to CHOP-like chemotherapy.
• Other subtypes like Angioimmunoblastic T-cell lymphoma (AITL) and PTCL-NOS have poor responses to standard therapies, necessitating novel approaches.

Conclusions:

• PTCL diagnosis requires expertise, with lower inter-observer agreement than for B-cell lymphomas.
• Risk stratification using subtype and prognostic indices is crucial for treatment planning.
• Tailored therapeutic strategies, including radiotherapy for specific subtypes and consideration of autotransplantation, are essential for improving outcomes in aggressive PTCL.