Peripheral T-Cell Lymphoma, Not Otherwise Specified: A Review of Current Disease Understanding and Therapeutic Approaches.
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), corresponds with a heterogeneous group of mature T-cell lymphomas. Recent gene expression profiling studies have identified at least two molecular subgroups (GATA3 and TBX2). Standard treatment and outcomes remain poor. High-dose chemotherapy with autologous stem cell transplantation is incorporated into primary therapy for young fit patients but remains ineffective for most and has not been tested in a randomized study. Several novel agents have been approved for use in relapsed/refractory PTCLs, and although response rates are modest for most, durable remissions have been reported. Selecting rationale combinations and incorporating predictive biomarkers will be important moving forward to improve outcomes in patients with PTCL.
Division of Hematology, Department of Hematology, Faculty of Medicine, Gordon and Leslie Diamond Health Care Centre, University of British Columbia, 2775 Laurel Street, 10th Floor, Vancouver, British Columbia V5Z 1M9, Canada; Faculty of Medicine, King Saud University, Riyadh 12372, Saudi Arabia.
Musa
M
Al-Zahrani
Department of Medical Oncology, British Columbia Cancer Agency, 600 West 10th Avenue, Vancouver, BC V5Z 4E6, Canada. Electronic address: ksavage@bccancer.bc.ca.
Kerry J
KJ
Savage
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D064592:Autografts; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism*; D050990:GATA3 Transcription Factor / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics / Q000378:metabolism* / Q000628:therapy*; D033581:Stem Cell Transplantation; D020825:T-Box Domain Proteins / Q000235:genetics / Q000378:metabolism*
D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D050990:GATA3 Transcription Factor; C494749:GATA3 protein, human; C097348:T-Box Domain Protein 2; D020825:T-Box Domain Proteins
CHOP; PTCL-NOS; Peripheral T-cell lymphoma, not otherwise specified