Department of Medicine, University of Nebraska Medical Center, Omaha, Nebraska.
Am J Hematol. 2013 Oct;88(10):910-8. doi: 10.1002/ajh.23536.
T-cell lymphomas make up approximately 10-15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia.
The diagnosis of aggressive peripheral T-cell lymphoma (PTCL) is usually made using the WHO classification. The ability of hematopathologists to reproducibly diagnosis aggressive PTCL is lower than for aggressive B-cell lymphomas, with a range of 72-97% for the aggressive PTCLs.
Patients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, PET scans appear to be useful as they are in aggressive B-cell lymphomas. The most commonly used prognostic index is the International Prognostic Index. The specific subtype of aggressive PTCL is an important risk factor, with the best survival seen in anaplastic large cell lymphoma-particularly young patients with the anaplastic lymphoma kinase positive subtype.
Anaplastic large cell lymphoma is the only subgroup to have a good response to a CHOP-like regimen. Angioimmunoblastic T-cell lymphoma has a prolonged disease-free survival in only ~20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL-NOS (not otherwise specified) is not one disease. Anthracycline containing regimens have disappointing results and a new approach is needed. NK/T-cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy containing regimens. Enteropathy associated PTCL and hepatosplenic PTCL are rare disorders with a generally poor response to therapy, although selected patients with enteropathy associated PTCL seem to benefit from intensive therapy.
T 细胞淋巴瘤约占淋巴恶性肿瘤的 10-15%。这些淋巴瘤的发病率在地理上有所不同，亚洲部分地区的发病率最高。
侵袭性外周 T 细胞淋巴瘤（PTCL）的诊断通常采用世界卫生组织（WHO）分类。血液病理学家能够准确诊断侵袭性 PTCL 的能力低于侵袭性 B 细胞淋巴瘤，侵袭性 PTCL 的诊断准确率为 72-97%。
采用 Ann Arbor 分类对侵袭性 PTCL 患者进行分期。尽管存在一定争议，但 PET 扫描似乎与侵袭性 B 细胞淋巴瘤一样有用。最常用的预后指数是国际预后指数（International Prognostic Index）。侵袭性 PTCL 的具体亚型是一个重要的危险因素，其中间变性大细胞淋巴瘤的生存情况最好，尤其是具有间变性淋巴瘤激酶阳性亚型的年轻患者。
间变性大细胞淋巴瘤是唯一对 CHOP 样方案有良好反应的亚组。血管免疫母细胞性 T 细胞淋巴瘤仅有约 20%的患者无疾病生存时间延长，但在缓解期进行自体移植的年轻患者似乎效果更好。PTCL-NOS（非特指型）不是一种疾病。含蒽环类药物的方案疗效不佳，需要新的治疗方法。局限于鼻和鼻旁窦的 NK/T 细胞淋巴瘤似乎最好采用含放疗的方案进行治疗。肠病相关 T 细胞淋巴瘤和肝脾 T 细胞淋巴瘤是罕见疾病，对治疗的反应普遍较差，尽管少数肠病相关 T 细胞淋巴瘤患者似乎受益于强化治疗。
