Pauwels P, Dal Cin P, Vlasveld L T, Aleva R M, van Erp W F, Jones D
Stichting PAMM, Catharina Ziekenhuis, Eindhoven, The Netherlands.
Am J Surg Pathol. 2000 Jun;24(6):882-8. doi: 10.1097/00000478-200006000-00016.
The pathogenesis of the hyaline vascular variant of Castleman's disease is currently unknown; however, vascular and dendritic cell proliferations are common in this disorder. We report a clonal karyotypic abnormality (46,XX,t(1;16) (p11;p11), del(7)(q21q22),del(8)(q12q22)) in 15 of 20 cells obtained after short-term stromal cultures of a typical case of hyaline vascular Castleman's disease (HVCD). There was no histologic, immunohistochemical, or genotypic evidence of a clonal lymphoid or plasma cell proliferation supporting origin of this aberration from the stromal component, possibly dendritic cells. We re-examined 15 previous cases of HVCD and identified a spectrum of dysplastic changes in the follicular dendritic cells (FDC) of atrophic lymphoid follicles, with some cases showing expansions of FDC networks by CD21 immunostaining. We propose that localized clonal proliferations of stromal elements, particularly follicular dendritic cells, occur in typical HVCD and likely explain the increased incidence of FDC sarcomas in these patients.
Castleman病透明血管型的发病机制目前尚不清楚；然而，血管和树突状细胞增殖在这种疾病中很常见。我们报告了1例典型透明血管型Castleman病（HVCD）短期基质培养后获得的20个细胞中有15个存在克隆性核型异常（46,XX,t(1;16) (p11;p11), del(7)(q21q22),del(8)(q12q22)）。没有组织学、免疫组化或基因型证据表明存在克隆性淋巴细胞或浆细胞增殖，支持这种异常起源于基质成分，可能是树突状细胞。我们重新检查了15例既往HVCD病例，发现萎缩性淋巴滤泡的滤泡树突状细胞（FDC）存在一系列发育异常变化，部分病例通过CD21免疫染色显示FDC网络扩张。我们提出，典型HVCD中存在基质成分尤其是滤泡树突状细胞的局限性克隆增殖，这可能解释了这些患者中FDC肉瘤发病率增加的原因。
