富于间质透明血管型Castleman病的病理形态、增生间质细胞组织起源和临床并发症关系探讨

Stroma-rich variant of hyaline.vascular Castleman＇s disease： a clinicopathologic and histogenetic study

目的对透明血管型Castleman病滤泡间区内间质细胞的病理形态、免疫表型以及组织来源进行深入研究，在此基础上，对间质细胞在疾病形成以及副肿瘤天疱疮发生过程中的作用进行初步探讨。方法选取透明血管型Castleman病23例，收集详细临床资料，分析组织病理形态，对全部病例进行免疫组织化学染色，抗体有CCL21、肌特异性肌动蛋白（MSA）、CD21、CD35、S-100蛋白和CD34。按照Danon等的标准进行分类，将滤泡间区占淋巴结面积大于50％的病例归入富于间质型，其余为普通型。结果23例中，富于间质型14例，普通型9例。富于间质型病例伴副肿瘤天疱疮11例，普通型无一例伴副肿瘤天疱疮，两者差异有统计学意义（P〈0．01）。滤泡间区内间质细胞免疫组织化学染色结果：普通型9例，CC121、MSA均为阳性，CD21、CD35、S-100蛋白、CD34均为阴性；富于间质型14例，CCL21阳性13例，MSA阳性13例，CD21、CD35、S-100蛋白、CD34均为阴性；组间差异无统计学意义（P〉0．05）。结论透明血管型Castleman病在病理上存在普通型和富于间质型两种表现，两者在副肿瘤天疱疮的发生率上存在显著差异。纤维母细胞性网状细胞是透明血管型Castleman病滤泡间区内最主要的间质细胞，其特征性的免疫表型为CCL21＋／MSA＋／CD34^-／CD21^-／S-100^-。富于间质型患者易于伴发副肿瘤天疱疮，可能与纤维母细胞性网状细胞的过度增生有关，详细的机制有待进一步研究。

Objective To study the histologic features and immunohistochemical findings of interfollicular stromal ceils in hyaline-vascular Castleman＇ s disease （HVCD） , and to explore the role of these stromal cells in the pathogenesis of this disease. Methods The clinical findings and microscopic features of 23 cases of HVCD cases were reviewed. Immunohistochemical study for CCL21, MSA, CD21, CD35, S-100 and CD34 was carried out. Results According to the criteria proposed by Danon et al. , stroma-rich variant of HVCD contained prominent interfollicular zone which occupied at least 50% of the lymph node area. In the current study, there were 14 cases of stroma-rich HVCD and 9 cases of conventional HVCD. Eleven of the stroma-rich HVCD had paraneoplastic pemphigus and contrastly, no pemphigus lesion obtained in all the 9 cases of conventional HVCD. The association between stromal cell hyperplasia and paraneoplastic pemphigus was statistically significant （ P 〈 0. 01 ）. In all the conventional HVCD cases studied,CCL21 and MSA were positive in the stromal cells. The stromal cells in 13 of the 14 cases of the stroma-rich HVCD were also positive for CCL21 and MSA,however, staining for CD21, CD35, S-100 and CD34 was negative in both groups. There was no statistical significance obtained （ P 〉 0. 05 ） between the differences of the staining results. Conclusions Stroma-rich HVCD and conventional HVCD represent two distinctive bistologic variants and have a different association with paraneoplastic pemphigus. Most of the stromal cells locating in the interfollicular areas are fibroblastic reticular cells in origin, with the immunophenotype as CCL21 ＋/MSA＋/CD34 -/CD21 -/S-100 -. The stromal cells proliferation correlate with the occurrence of paraneoplastic pemphigus, nevertheless, more cases are expected for a further study of the underlying pathogenesis.