TY - CHAP M1 - Book, Section TI - Nodal Peripheral T-Cell Lymphoma A1 - Nair, Ranjit A1 - Vega, Francisco A1 - Iyer, Swaminathan P. A2 - Kantarjian, Hagop M. A2 - Wolff, Robert A. A2 - Rieber, Alyssa G. Y1 - 2022 N1 - T2 - The MD Anderson Manual of Medical Oncology, 4e AB - KEY CONCEPTSNodal 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. SN - PB - McGraw Hill Education CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1190833189 ER -