Division of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada.
Crit Rev Oncol Hematol. 2011 Sep;79(3):321-9. doi: 10.1016/j.critrevonc.2010.07.007. Epub 2010 Aug 10.
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) does correspond to a heterogeneous group of nodal and extranodal mature T-cell lymphomas, with a low prevalence in Western countries. PTCL-NOS accounts for about 25% of all PTCL, which represent over 15% of all lymphomas. In the lymph node, PTCL-NOS shows paracortical or diffuse infiltrates with effacement of the normal architecture, with a broad cytological spectrum and a frequently observed inflammatory background. Some morphological variants include: lymphoepithelioid or Lennert's type, T-zone, and follicular. PTCL-NOS is characterized by an aberrant T-cell phenotype, with frequent loss of CD5 and CD7. A CD4+/CD8- phenotype predominates in nodal cases. CD4/CD8 +/+ or -/- is at times seen, as is CD8, CD56 and cytotoxic granule expression. Ki-67 rate is typically high. TCR β-chain is usually expressed; TCR genes are most often clonally rearranged. PTCL-NOS typically occurs in adults (median age 55-60 years), with a higher prevalence in males. It presents more often as disseminated disease, occasionally with eosinophilia, pruritis or hemophagocytic syndrome. Patients often have B symptoms, generalized lymphadenopathy, bone marrow infiltration, and extranodal involvement, with high or high-intermediate IPI score in 50-70% of cases. Prognosis is poor, with a 5-year OS of 20-30%. Some variables, like ST2(L), CXCR5, CXCR3, EBV infection, cytotoxic granule expression, high proliferative index, NF-κB expression, were proposed as prognostic indicators, but the IPI score, and its variant called PIT, remains the most effective prognostic factor. Patients with PTCL-NOS should be treated with anthracycline-containing chemotherapy, followed by radiotherapy in cases of stage I-II disease. This strategy is associated with an overall response rate higher than 60%, but the 5-year overall survival is only 20-30%. Upfront high-dose chemotherapy supported by autologous or allogeneic SCT is an investigational approach, with a 4-year overall survival of about 40%. Patients with chemosensitive relapse respond favorably to high-dose chemotherapy and ASCT, with long-term survival rates of 35-45%. Graft-versus-lymphoma effect following allogeneic SCT has been observed; and reduced intensity conditioning emerges as an attractive strategy for frail patients. Most patients with PTCL-NOS are enrolled in prospective trials to explore new approaches, and new agents, like gemcitabine, alemtuzumab and pralatrexate, are being investigated.
外周 T 细胞淋巴瘤，非特指型（PTCL-NOS）的确对应于一组异质性的结内和结外成熟 T 细胞淋巴瘤，在西方国家的发病率较低。PTCL-NOS 约占所有 PTCL 的 25%，占所有淋巴瘤的 15%以上。在淋巴结中，PTCL-NOS 表现为具有正常结构破坏的副皮质或弥漫性浸润，具有广泛的细胞学谱和经常观察到的炎症背景。一些形态学变异包括：淋巴上皮样或 Lennert 型、T 区和滤泡型。PTCL-NOS 的特征是异常的 T 细胞表型，常伴有 CD5 和 CD7 的缺失。结内病例中以 CD4+/CD8-表型为主。有时可见 CD4/CD8++/--或 CD8、CD56 和细胞毒性颗粒表达。Ki-67 率通常较高。TCRβ链通常表达；TCR 基因通常克隆性重排。PTCL-NOS 通常发生在成年人（中位年龄 55-60 岁），男性患病率较高。它更多地表现为播散性疾病，偶尔伴有嗜酸性粒细胞增多、瘙痒或噬血细胞综合征。患者常有 B 症状、全身淋巴结肿大、骨髓浸润和结外累及，50-70%的病例具有高或高-中 IPI 评分。预后较差，5 年 OS 为 20-30%。一些变量，如 ST2(L)、CXCR5、CXCR3、EBV 感染、细胞毒性颗粒表达、高增殖指数、NF-κB 表达，被提出作为预后指标，但 IPI 评分及其变体 PIT 仍然是最有效的预后因素。PTCL-NOS 患者应接受含蒽环类药物的化疗，然后在 I-II 期疾病患者中进行放疗。这种策略与高于 60%的总缓解率相关，但 5 年总生存率仅为 20-30%。在自体或异基因 SCT 支持下进行的大剂量化疗是一种探索性方法，4 年总生存率约为 40%。对化疗敏感的复发患者对大剂量化疗和 ASCT 反应良好，长期生存率为 35-45%。在异基因 SCT 后观察到移植物抗淋巴瘤效应；并且强度降低的调理似乎是虚弱患者的一种有吸引力的策略。大多数 PTCL-NOS 患者都被纳入前瞻性试验中以探索新的方法，并且正在研究新的药物，如吉西他滨、阿仑单抗和普拉曲沙。
