These malignancies arise through acquired somatic alterations in T-lymphocyte or NK-cell lineages. No germline genes are catalogued for this umbrella entity in the current dataset. The molecular basis varies by subtype and is not fully characterized at the class level.

Diagnosis requires histopathological evaluation of biopsy tissue with immunohistochemical and flow cytometric characterization of the T-cell or NK-cell lineage. Molecular and cytogenetic studies assist in subtype classification. Precise diagnostic criteria depend on the specific subtype under evaluation.

Two agents carry FDA approval within this disease class. Belinostat (BELEODAQ), a histone deacetylase inhibitor, received FDA approval on July 3, 2014. Brentuximab vedotin (ADCETRIS), an antibody-drug conjugate, received FDA approval on August 19, 2011 for CD30-expressing subtypes. Treatment planning depends on subtype, disease stage, and individual factors and is coordinated by a multidisciplinary oncology team.

Outcomes vary substantially by subtype and disease extent. The majority of subtypes follow an aggressive clinical course. Prognosis is influenced by specific subtype, extent of disease at diagnosis, and response to therapy.

ClinicalTrials.gov lists 81 active studies. A Phase 3 trial (NCT06561048) is evaluating soquelitinib versus standard of care in relapsed or refractory PTCL. Additional studies are examining autologous hematopoietic cell transplantation, combination chemotherapy, and CAR-T cell approaches targeting CD30 and CCR4.