X. Challenges and future directions in peripheral T‐cell lymphoma

Treatment paradigms for peripheral T-cell lymphomas (PTCLs) have been modelled after B-cell lymphomas; however, cyclophosphamide,doxorubicin, vincristine, and prednisone (CHOP)-(like) chemotherapy remains largely ineffective. The clear exception is anaplastic lymphoma kinase positive anaplastic large cell lymphoma (ALCL) (ALK+ALCL), which has high cure rates with CHOP. A subset of ALK-negative (ALK ) ALCL harbouring a DUSP22 rearrangement has a similarly favourable outcome. Limited studies support that a ‘one size fits all’ approach is not optimal for all PTCLs, and there is rationale for tailored therapy for some subtypes. Recently, a number of novel agents have been explored in PTCLs, and four have been Food and drug association (FDA)-approved (pralatrexate, brentuximab vedotin, romidepsin and belinostat) for relapsed and refractory disease and are being explored in the front-line setting. Further, there have been several recent advances in the understanding of the biology of PTCLs based on molecular profiling and next-generation sequencing, which may shed light into altered pathways to target therapeutically.