Walter Reed Army Medical Center Washington, DC, USA.
Oncology (Williston Park). 2010 Jan;24(1):94-100.
Peripheral T-cell lymphomas (PTCLs) are uncommonly encountered malignancies in the United States, and hepatosplenic T-cell lymphoma (HSTCL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and enteropathy-type T-cell lymphoma (ETTCL) are rare subtypes of PTCLs that often present with primarily extranodal disease. Despite the fact that these tumors have distinct clinical and pathologic features, they are often diagnosed after significant delay. The combination of delay in diagnosis with ineffective therapies has resulted in a poor prognosis in most cases. Techniques that identify T-cell receptor gene rearrangements and flow cytometry that can identify characteristic immunophenotypes have guided our understanding of the underlying cell of origin of these rare PTCLs. As knowledge regarding the biology of these lymphomas increases alongside the development of newer therapeutics with novel mechanisms, clinicians must accordingly improve their familiarity with the clinical settings in which these rare malignancies arise as well as the pathologic features that make them unique.
外周 T 细胞淋巴瘤（PTCLs）在美国是一种罕见的恶性肿瘤，肝脾 T 细胞淋巴瘤（HSTCL）、皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）和肠病型 T 细胞淋巴瘤（ETTCL）是罕见的 PTCL 亚型，通常表现为主要结外疾病。尽管这些肿瘤具有明显的临床和病理特征，但通常在明显延迟后才被诊断出来。诊断延迟与无效治疗相结合，导致大多数情况下预后不良。识别 T 细胞受体基因重排的技术和可识别特征性免疫表型的流式细胞术指导了我们对这些罕见的 PTCL 起源细胞的理解。随着对这些淋巴瘤生物学的认识不断增加，以及具有新型机制的新型治疗方法的发展，临床医生必须相应地提高他们对这些罕见恶性肿瘤出现的临床环境以及使它们具有独特特征的病理特征的熟悉程度。
