Division of Hematology/Oncology, University of California Irvine, Long Beach, CA.
Division of Hematology/Oncology, University of Virginia, Charlottesville, VA.
Semin Hematol. 2021 Apr;58(2):95-102. doi: 10.1053/j.seminhematol.2021.03.001. Epub 2021 Mar 19.
Despite the remarkable improvements in the treatment and outcome of patients with aggressive B-cell lymphoma, the peripheral T-cell lymphomas (PTCL) continue to carry a poor prognosis with the presently available treatment options. The PTCL are very rare diseases that account for only 10,000 to 15,000 new cases per year in the United States. The World Health Organization's 2016 classification describes 29 distinct subtypes of PTCL, thus making these both rate and incredibly heterogenous. The 2 most common forms of PTCL, for example, peripheral T-cell lymphoma-not otherwise specified and angioimmunoblastic T-cell lymphoma , have an incidence of only 2500 and 1800 cases per year respectively, in the United States.
尽管侵袭性 B 细胞淋巴瘤患者的治疗和预后取得了显著改善，但外周 T 细胞淋巴瘤（PTCL）的现有治疗选择仍预后不良。PTCL 是非常罕见的疾病，在美国每年仅有 10,000 至 15,000 例新发病例。世界卫生组织 2016 年的分类描述了 29 种不同的 PTCL 亚型，因此这些疾病不仅发病率低，而且异质性极高。例如，在美国，最常见的两种 PTCL 形式，即未特指的外周 T 细胞淋巴瘤和血管免疫母细胞性 T 细胞淋巴瘤，每年的发病率分别仅为 2500 例和 1800 例。
