Al-Zahrani Musa, Savage Kerry J
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.
Department of Medical Oncology, British Columbia Cancer Agency, 600 West 10th Avenue, Vancouver, BC V5Z 4E6, Canada.
Hematol Oncol Clin North Am. 2017 Apr;31(2):189-207. doi: 10.1016/j.hoc.2016.11.009.
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.
外周T细胞淋巴瘤，非特指型（PTCL-NOS），对应一组异质性的成熟T细胞淋巴瘤。最近的基因表达谱研究已鉴定出至少两个分子亚组（GATA3和TBX2）。标准治疗方法及疗效仍然欠佳。自体干细胞移植的大剂量化疗被纳入年轻健康患者的一线治疗，但对大多数患者仍无效，且尚未在随机研究中得到验证。几种新型药物已被批准用于复发/难治性PTCL，尽管大多数药物的缓解率一般，但已有持久缓解的报道。选择合理的联合方案并纳入预测性生物标志物对于改善PTCL患者的预后将至关重要。
