CXCL13、CD10和bcl-6在血管免疫母细胞性T细胞淋巴瘤的诊断及鉴别诊断中的作用

Expressions of CXCL13, CD10 and bcl-6 in angioimmunoblastic T-cell lymphoma and peripheral T- cell lymphoma, not otherwise specified

目的探讨CXCL13、CD10、bcl-6等标志物在血管免疫母细胞性T细胞淋巴瘤（AITL）的诊断和鉴别诊断中的作用。方法对四川大学华西医院病理科1990年1月至2008年1月诊断的115例AITL、30例非特指外周T细胞淋巴瘤（PTCL，NOS）和30例以副皮质区增生为主的反应性增生（RH）进行回顾性分析。按2008版WHO关于淋巴造血组织肿瘤分类进行组织学分型，采用9种抗原标志物的免疫组织化学（SP法）染色及TCR-γ基因重排检测。结果（1）7．8％（9／115）的AITL、6．7％（2／30）的PTCL，NOS和83．3％（25／30）的RH病例观察到生发中心；98．3％（113／115）的AITL、63．3％（19／30）的PTCL，NOS和76．7％（23／30）的RH病例观察到显著血管增生。（2）CXCL13、CD10、bcl-6在RH病例的表达局限在生发中心，在AITL的表达率分别为96．5％（111／115）、50．4％（58／115）和78．3％（90／115），在PTCL，NOS的表达率分别为26．7％（8／30）、3．3％（1／30）和3．3％（1／30），以上三个标记在两种淋巴瘤的表达率差异均具有统计学意义。115例AITL病例均见到滤泡外不规则分布的CD21阳性的滤泡树突状细胞网（FDC）。TCR．1基因克隆性重排在AITL中检出率为83％（83／100）。结论AITL是一种来源于生发中心辅助性T细胞（TFH）的高度侵袭性肿瘤，CXCL13、CD10、bcl-6是AITL诊断和鉴别诊断有用标志物。

Objective To study the value of immunomarkers CXCL13, CD10, bcl-6 in pathologic diagnosis of angioimmunoblastic T-cell lymphoma （AITL）. Methods One hundred and fifteen cases of AITL, 30 cases of peripheral T-cell lymphoma, not otherwise specified （PTCL, NOS） and 30 cases of reactive lymph nodes with paracortical hyperplasia （RH） encountered during the period from January, 1990 to January, 2008 were retrieved from the archival files of the Department of Pathology, West China Hospital of Sichuan University, China. The morphologic features were reviewed and compared. Immunohisto- chemical study was performed by SP method for CXCL13, CD10, bcl-6, CD21, CD3e, CD3, CD45RO, CD20 and Ki-67. TCR-γ gene rearrangement study was also carried out. Results Regressed follicles were evident in 7. 8% （9/115） of AITL cases, 6. 7% （2/30） of PTCL, NOS cases and 83.3% （25/30） of RH cases, respectively. A marked increase of number of arborizing venules was shown in 98. 3 % （ 113/115） of AITL cases, 63.3% （19/30） of PTCL, NOS cases and 76.7% （23/30） of RH cases, respectively. In lymph nodes with paracortical hyperplasia, the expression of CXCL13, CD10 and bcl-6 were restricted to the germinal centers. In AITL, 96. 5% （111/115） of cases showed CXCL13 expression, in contrast to 26. 7% （8/30） of PTCL, NOS. Expression of CD10 and bcl-6 were found in the neoplastic cells in 50.4% （58/115） and 78.3% （90/115） of AITL, and 3.3% （1/30） and 3.3% （1/30） of PTCL, NOS, respectively. Irregular meshworks of CD21-positive follicular dendritic cells were found in all the AITL cases. Clonal TCR-γ rearrangement was detected in 83% （83/100） of the AITL cases. Conclusions AITL is a type of lymphoma originated from the follicular helper T cells. Detailed morphologic assessment and use of immunohistochemical markers are essential for accurate diagnosis.