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Clinicopathological Study of 30 Cases of Peripheral T-cell Lymphoma with Hodgkin and Reed-Sternberg-like B-cells from Japan.

30例来自日本的具有霍奇金样及HRS样B细胞的外周T细胞淋巴瘤临床病理研究

Eladl AE,Satou A,Elsayed AA,Suzuki Y,Kato S,Asano N,Nakamura S

Abstract

The presence of Hodgkin and Reed-Sternberg (HRS)-like B-cells in peripheral T-cell lymphoma (PTCL) is rare and its clinicopathological features still remain unclear. Here, we describe 30 cases of PTCL with HRS-like B-cells from Japan. Twenty-three cases (77%) presented evidence of follicular T-helper phenotype (TFH) derivation: 12 were angioimmunoblastic T-cell lymphoma and 11 PTCL with TFH phenotype (PTCL-TFH). The remaining seven cases were diagnosed as PTCL, not otherwise specified (PTCL-NOS). Epstein-Barr virus (EBV) reactivation was detected in 25 cases (83%), but HRS-like B-cells were EBER in only 20 cases (67%). The median age at diagnosis was 77 years (range, 39-91 y), including 24 patients (80%) were older than 60 years of age. Most of the patients presented at an advanced clinical stage and were associated with higher risk according to the International Prognostic Index. The 3-year overall and progression-free survival rates were 44% and 27%, respectively. No significant clinicopathological differences were detected between PTCL-TFH, PTCL-NOS and the angioimmunoblastic cases. Cases with EBER HRS-like B-cells were associated with inferior overall and progression-free survival compared to those with EBER HRS-like B-cells, but the difference was not significant. In conclusion, HRS-like B-cells were found in a subset of T-cell lymphomas, especially in association with the TFH phenotype and EBV reactivation. These cells have a tendency to affect elderly patients and to be associated with advanced clinical stages and dismal prognosis. The EBV status of HRS-like B-cells does not seem to affect the clinicopathological features of this group of PTCLs.

摘要

外周T细胞淋巴瘤(PTCL)出现霍奇金样及HRS样B细胞比较罕见,其临床病理特征仍不明确。我们在本文中描述了30个来自日本的具有HRS样B细胞的PTCL病例。23例(77%)表达了滤泡辅助T表型(TFH):12例为血管免疫细胞性T细胞淋巴瘤,11例为伴有TFH表型的PTCL(PTCL-TFH),其余7个病例诊断为PTCL,非特指(PTCL-NOS)。在25个病例(83%)发现了EB病毒(EBV)的激活,但只有20个病例(67%)HRS样B细胞EBER检测阳性。患者中位年龄77岁(39-91岁),包括24名60岁以上患者(80%)。大多数患者临床分期高,参照国际预后指数属于高风险。3年总体生存率和无进展生存率分别为44%和27%,在PTCL-TFH、PTCL-NOS和血管免疫细胞性淋巴瘤病例之间没有发现明显的临床病理差异。相比之下,具有EBER HRS样B细胞的病例整体生存率和无进展生存率更低,但差异并不显著。总之,在T细胞淋巴瘤的亚型中发现有HRS样B细胞,尤其是与TFH表型和EBV激活相关的T细胞淋巴瘤。这些细胞倾向于侵犯老年患者,与临床分期高和预后不良有关。HRS样B细胞的EBV状态似乎并不影响这组PTCLs的临床病理特征。


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