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  • Nodal peripheral T-cell lymphomas (PTCLs) are categorized into three basic subtypes: systemic anaplastic large-cell lymphoma (sALCL) anaplastic lymphoma kinase ALK (+) and ALK (-), angioimmunoblastic T-cell lymphoma; and peripheral T-cell lymphoma not otherwise specified (PTCL-NOS).

  • The prognosis for patients with nodal PTCLs remains suboptimal compared with its aggressive B cell counterparts despite the use of aggressive chemotherapy. The introduction of novel agents such as CD30(+) antibody–drug conjugates and histone deacetylase inhibitors have brought unprecedented enthusiasm in bench to bedside research for this disease.

  • It is recently recognized that angioimmunoblastic T–cell lymphoma has its cell of origin from CD4+ T-follicular helper (TFH) cells and as a result a third of the PTCL-NOS are more aptly diagnosed as angioimmunoblastic T-cell lymphoma because of the expression of TFH markers.

  • Understanding of the genomic landscape has recently helped to identify better prognostic markers. Whereas the presence of ALK or DUSP-22 in sALCL is associated with favorable prognosis, expression of GATA-3 or GATA-3 target genes in PTCL-NOS is associated with a worse prognosis.

  • Except for ALK (+) ALCL and ALK (-) ALCL with DUSP22 rearrangement, patients with PTCL generally have a chemoresistance, short-term remission, and early relapse when treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or CHOP-like chemotherapy.

  • Combination therapies using novel agents holds the future to improve clinical response and outcomes in both frontline and relapsed or refractory disease. Whereas BV-CHP (brentuximab vedotin plus cyclophosphamide, doxorubicin, prednisone) is currently the standard of care treatment in patients with CD30(+) PTCLs, Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), with or without the addition of etoposide, is commonly used in those with CD30(-) PTCLs.


Peripheral T-cell lymphoma (PTCL) comprises a rare and heterogeneous group of mature T- and natural killer (NK)-cell neoplasms with diverse clinical presentation, response to treatment, and prognosis.1 PTCLs have historically been a challenging disease because of their rarity, often grouped by their shared immunophenotype of neoplastic T-cells, despite the biologically differences and lack of standard treatment approaches.2 Getting a second opinion and reviews by expert hematopathologists improve the accuracy of subtyping, and referral to academic or specialty centers is recommended. Even though there is no current standard of care for patients with PTCL, first-line approaches still revolve around anthracycline-based therapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or CHOP-like combination chemotherapies. Although many patients initially respond to induction chemotherapy, responses are often brief, and many patients relapse or become refractory to treatment. The introduction of agents based on the novel targets such as CD30 and brentuximab vedotin (BV) has given us tremendous hope recently leading to the only Food and Drug Administration (FDA)–approved chemotherapy combination for first-line treatment. Significant advances in our understanding of this disease biology have also led to revisions in the classification of mature T-cell and NK-cell neoplasms and introduction of provisional entities. According to the 2016 World Health Organization classification (WHO) classification of lymphoid neoplasms, based on morphologic, immunophenotypic, molecular, and clinical features, ...

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