Overview
Peripheral T-cell lymphoma (PTCL) encompasses a heterogeneous group of non-Hodgkin lymphomas originating from mature T or natural killer (NK) cells, often presenting with diverse morphological and immunophenotypic features 1.Diagnosis
Immunophenotyping: Essential for classification; includes evaluation of CD3, CD5, CD7, CD30 expression, and others to distinguish subtypes 1.
EBV Testing: Epstein-Barr virus (EBV) EBER1/2 hybridization recommended to assess potential association with certain subtypes 1.
Morphological Assessment: Critical for identifying broad spectrum of presentations within PTCL subtypes 1.
WHO Classification: Cases reviewed and classified according to the WHO classification system for lymphoid neoplasms 1.Management
First-Line Treatment: Chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) are commonly used, though efficacy varies by subtype 1.
Subtype-Specific Approaches: Certain subtypes like enteropathy-associated T-cell lymphoma may require tailored treatments beyond standard CHOP 1.
Adjunctive Therapies: Rituximab may be considered in specific cases, particularly those with B-cell markers or EBV positivity 1.
Clinical Trials: Encouraged for patients with refractory or relapsed disease due to limited evidence for standard treatments 1.Special Populations
Elderly Patients: Treatment strategies often adjusted for comorbidities and performance status; less robust evidence exists for specific elderly protocols 1.
Comorbidities: Management must consider coexisting conditions, potentially influencing treatment choice and intensity 1.Key Recommendations
Utilize comprehensive immunophenotyping and morphological assessment for accurate PTCL classification (Evidence: Moderate 1).
Incorporate EBV testing to guide management decisions, especially in subtypes with potential viral associations (Evidence: Moderate 1).
Tailor first-line chemotherapy based on PTCL subtype, considering CHOP as a foundational regimen with modifications as needed (Evidence: Expert opinion 1).References
1 Kluin PM, Feller A, Gaulard P, Jaffe ES, Meijer CJ, Müller-Hermelink HK et al.. Peripheral T/NK-cell lymphoma: a report of the IXth Workshop of the European Association for Haematopathology. Histopathology 2001. link