T细胞淋巴瘤
T-Cell Lymphoma
作者信息
Varghese Merryl T., Alsubait Saud
机构信息
Michigan State University
出版信息
The lymphoid system is the pillar of the human immune response. It comprises innate immunity covered by NK cells and adaptive immunity by the B and T-cells. Non-Hodgkin lymphoma (NHL) is a disorder of these immune cells. The understanding of these cells from their formation to maturation is necessary for the diagnosis and management of this disease spectrum. T-cell lymphomas consist of a wide variety of uncommon diseases that can be indolent or aggressive and comprise 12% of all NHL. Since NK-cells are closely related to the T-cells, their malignancies are also included in the classification. WHO released a revised lymphoma classification in 2016, which broadly divided T-cell/NK-cell lymphomas as the precursor and mature T-cell neoplasms. The mature subgroup is further divided into leukemic, nodal, extranodal, and cutaneous. NHL can also be divided into indolent or aggressive diseases. Indolent lymphomas are characterized by a long disease course and are generally resistant to standard chemotherapy, whereas aggressive disease usually has an acute presentation with B symptoms and rapid progression. Cutaneous T-cell lymphoma (CTCL) is considered indolent, whereas peripheral mature lymphomas (PTCL), which include the rest, are considered aggressive. Two-thirds of the CTCLs are comprised of mycosis fungoides (MF) and Sezary syndrome (SS). PTCL-NOS (not otherwise specified) is the most common among the PTCL, followed by anaplastic large cell lymphoma (ALCL) and angioimmunoblastic T-cell lymphoma (AITL).
淋巴系统是人体免疫反应的支柱。它包括由自然杀伤细胞(NK细胞)介导的固有免疫和由B细胞及T细胞介导的适应性免疫。非霍奇金淋巴瘤(NHL)是这些免疫细胞的一种紊乱疾病。了解这些细胞从形成到成熟的过程对于诊断和管理这一疾病谱至关重要。T细胞淋巴瘤由多种罕见疾病组成,这些疾病可以是惰性的或侵袭性的,占所有NHL的12%。由于NK细胞与T细胞密切相关,它们的恶性肿瘤也包括在分类中。世界卫生组织(WHO)在2016年发布了修订后的淋巴瘤分类,将T细胞/NK细胞淋巴瘤大致分为前驱型和成熟T细胞肿瘤。成熟亚组进一步分为白血病型、淋巴结型、结外型和皮肤型。NHL也可分为惰性或侵袭性疾病。惰性淋巴瘤的特点是病程长,通常对标准化疗耐药,而侵袭性疾病通常急性起病,伴有B症状且进展迅速。皮肤T细胞淋巴瘤(CTCL)被认为是惰性的,而包括其他类型的外周成熟T细胞淋巴瘤(PTCL)则被认为是侵袭性的。三分之二的CTCL由蕈样肉芽肿(MF)和赛塞里综合征(SS)组成。PTCL-NOS(未另行指定)是PTCL中最常见的类型,其次是间变性大细胞淋巴瘤(ALCL)和血管免疫母细胞性T细胞淋巴瘤(AITL)。