Castleman病的病变细胞类型鉴定

Castleman病是1954年发现的一种罕见的淋巴增殖性疾病，迄今六十多年来，该病的病变细胞类型一直未被确定。我们在前期偶然发现，Castleman病的病变细胞可能是一小群携带体细胞突变的“肿瘤样”间质细胞，这为阐明Castleman病变细胞类型这个难题提供了明确的方向。通过进一步研究发现，这些“肿瘤样”间质细胞表达NG2和CD146（周细胞pericyte的标志物），而不表达CD31（血管内皮细胞标志物）和CD21（滤泡树突状细胞标志物）。因此我们推测：Castleman病的病变细胞可能来源于血管周围的周细胞。本项目将通过鉴定细胞类型和模拟发病过程两方面验证上述假说。项目的顺利实施，将明确Castleman病的病变细胞类型并建立新的发病机制，为临床治疗方案的选择提供重要的实验依据。

Castleman disease was first described in a case report by Castleman in 1954, which is an uncommon lymphoproliferative disorder that encompasses a diverse group of clinical syndromes. Whether Castleman disease is a malignant or an infectious disease has long been a matter of intense debate. Until now, the cell type responsible for driving Castleman disease pathogenesis has not been definitively identified. In the previous study, we indentified that neoplastic cells in Castleman disease might be these non-hematopoietic stromal cells with somatic mutations, which provides a clear direction for preliminary solving the problem that has plagued researchers for decades. Further studies indicated that these neoplastic stromal cells with somatic mutations were NG2+ and CD146+ (pericyte cell markers), CD31- (marker of vascular endothelial cells) and CD21- (follicular dendritic cell marker). Therefore, we assume that the cell type responsible for driving Castleman disease pathogenesis may be pericyte. To verify our hypothesis, we will further identify the cell type of these neoplastic stromal cells at first, and then try to establish an animal model to elucidate the pathogenesis of Castleman disease. The identification of cell type responsible for driving Castleman disease pathogenesis may thus pave the way for novel and powerful therapeutics for the treatment of Castleman disease, and which is very important in both theoretical researches and clinical application.