The aggressive peripheral T-cell lymphomas: 2017
The aggressive peripheral T-cell lymphomas: 2017
复制标题
DOI：
10.1002/ajh.24791
复制
发表时间：
2017-07-01
影响因子：
12.8
通讯作者：
Armitage, James O.
中科院分区：
文献类型：
--
作者：
Armitage, James O.
背景：T细胞淋巴瘤约占淋巴系统恶性肿瘤的10%-15%。这些淋巴瘤的发病率因地理位置而异，亚洲部分地区发病率最高。诊断：侵袭性外周T细胞淋巴瘤（PTCL）的诊断通常使用世界卫生组织分类。血液病理学家可重复诊断侵袭性PTCL的能力低于侵袭性B细胞淋巴瘤，侵袭性PTCL的范围为72%-97%。风险分层：侵袭性PTCL患者采用安阿伯分类法进行分期。虽然有些争议，正电子发射断层扫描似乎是有用的，因为它们是在侵略性B细胞淋巴瘤。侵袭性PTCL的特定亚型是一个重要的危险因素，在间变性大细胞淋巴瘤中观察到最好的生存率，特别是间变性淋巴瘤激酶阳性亚型的年轻患者。风险适应性治疗：间变性大细胞淋巴瘤是唯一一个对CHOP样方案有良好反应的亚组。血管免疫母细胞性T细胞淋巴瘤只有20%的患者有延长的无病生存期，但年轻患者在缓解期接受自体移植似乎更好。PTCL-未另行说明不是一种疾病。含蒽环类药物的治疗方案效果令人失望，需要一种新的方法。局限于鼻和鼻窦的自然杀伤/T细胞淋巴瘤似乎最好用含放射治疗的方案治疗，大多数患者都治愈了。肠病相关性PTCL和肝脾PTCL是罕见疾病，通常对治疗反应较差，尽管选定的肠病相关性PTCL患者似乎从强化治疗中获益。
Background: 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.Diagnosis: The diagnosis of aggressive peripheral T-cell lymphoma (PTCL) is usually made using the World Health Organization classification. The ability of hematopathologists to reproducibly diagnose aggressive PTCL is lower than that for aggressive B-cell lymphomas, with a range of 72%-97% for the aggressive PTCLs. Risk Stratification: Patients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, positron emission tomography scans seem to be useful as they are in aggressive B-cell lymphomas. 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.Risk-Adapted Therapy: 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 similar to 20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL-not otherwise specified is not one disease. Anthracycline-containing regimens have disappointing results, and a new approach is needed. Natural killer/T-cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy-containing regimens and the majority of patients are cured. 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.