Peripheral T-cell lymphoma with follicular helper of T cell phenotype of Waldeyer′s ring: a clinicopathological and genetic study of eight cases
10.3760/cma.j.cn112151-20200213-00093
- VernacularTitle:韦氏环伴滤泡辅助T细胞表型的外周T细胞淋巴瘤八例临床病理学及遗传学分析
- Author:
Dandan ZHANG
1
;
Pan LI
;
Peizhu HU
;
Guannan WANG
;
Wugan ZHAO
;
Yanping ZHANG
;
Wencai LI
Author Information
1. 郑州大学第一附属医院病理科 450052
- Keywords:
Lymphoma, T-cell, peripheral;
Immunoblastic lymphadenopathy;
Pathology, clinical;
Genes;
Waldeyer′s ring
- From:
Chinese Journal of Pathology
2020;49(7):686-692
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To study the clinicopathologic and genetic features of Waldeyer′s ring peripheral T-cell lymphoma with follicular helper T cell immunophenotypes (wPTCL-TFH), with comparison to the nodal peripheral T-cell lymphoma with TFH immunophenotypes (nPTCL-TFH) and angioimmunoblastic T-cell lymphoma (AITL), as to know this rare tumor better.Methods:The clinical data, histopathology features, EBV positivity, T cell clonality and IDH2 R172 gene mutation in 8 cases of wPTCL-TFH were collected at the First Affiliated Hospital of Zhengzhou University from December 2015 to April 2019, and analyzed by immunohistochemistry, in situ hybridization, TCR gene rearrangement (BIOMED-2) and Sanger sequencing.Follow-up data were obtained by telephone. Results:There were 6 males and 2 females with a median age of 62.5 years (age ranging from 30 to 75 years). All patients had neither fever nor skin manifestations, but were all found mucosa thickened or mass of waldeyer′s ring with multiple lymph nodes enlarged by PET-CT/CT scans. Five of the 7 patients were at advanced stages (Ⅲ/Ⅳ stage). Microscopically, the mucosa was infiltrated diffusely and characteristically by numerous small-medium sized lymphocytes, lacking polymorphous inflammatory background and extra-follicular expansion of follicular dendritic cell networks (FDC networks). The clear T cells presented in 5 cases. Ulcers on mucosal surfaces (6 cases) and local-extensive loss of intramucosal glands (7 cases) were commonly noted. Granulomas composed of epithelioid histiocytes were observed in 2 cases. Immunohistochemically, all the tumor cells expressed CD4 and at least 2 types of follicular helper of T cell (TFH) markers: PD-1 (8/8), bcl-6 (8/8), CXCL13 (7/8) and CD10 (1/8). Most of the cases (6 cases) expressed CD30. EBV positive appeared in 4 cases. All 8 cases were T cell monoclonal. IDH2 R172 were wild-type in 6 cases. One patient died at the follow-up time on 18 months; the other 7 survived (the follow-up time varied from 3 to 10 months). Conclusions:wPTCL-TFH is rare, and its clinicopathological features are similar to nPTCL-TFH which may be the manifestation of the same disease at different stage, and partly overlapped with AITL. The differential diagnosis from PTCL-NOS is necessary and comprehensive analyses of clinical, morphological, immunohistochemical and genetic features can help make a correct diagnosis.
