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目的探讨Castleman病克隆性重排检测及其在鉴别诊断中的应用。方法收集组织病理学确诊的Castleman病38例,利用根据BIOMED-2引物体系设计的Identi Clone系列淋巴瘤基因重排检测试剂盒及Genescanning法进行免疫球蛋白(Ig)和T细胞受体(TCR)基因克隆性重排检测及结果分析。结果 38例CD病理组织学分型为透明血管型25例,浆细胞型9例及混合型4例。根据临床病变累及部位为局限型21例,多中心型17例。克隆性分析结果,10例CD克隆性重排阳性,其中6例为Ig克隆性重排,2例为TCR基因重排,2例为Ig和TCR基因双重排。2例MCD为Ig H和/或Igk单克隆重排,随访13及21个月后发展为滤泡性淋巴瘤及弥漫大B细胞淋巴瘤。结论大多数CD的淋巴细胞为多克隆性起源,Ig和/或TCR基因发生克隆性重排提示单克隆性增生具有恶性转化趋势和潜能,对推测预后有一定帮助。
Objective To investigate the clonal rearrangement of Castleman’s disease and its application in differential diagnosis. Methods Forty-eight patients with histopathologically diagnosed Castleman’s disease were collected. Immunoglobulin (Ig) and T cell receptor (TCR) receptor were detected by Identi Clone series lymphoma gene rearrangement assay kit and Genescanning method according to BIOMED-2 primer system. Gene cloning rearrangement detection and analysis of results. Results 38 cases of CD histopathological classification of 25 cases of clear blood vessels, plasma cell type in 9 cases and mixed type in 4 cases. According to clinical lesions involving the limited type of 21 cases, 17 cases of multi-center type. Clonal analysis showed that 10 cases of CD clonal rearrangements were positive, of which 6 cases were Ig clonal rearrangements, 2 cases were TCR gene rearrangements and 2 cases were Ig and TCR gene double rearrangements. Two of the MCDs were monoclonal IgH and / or Igk rearrangements that developed follicular lymphoma and diffuse large B-cell lymphoma at 13 and 21 months of follow-up. Conclusion Most of the CD lymphocytes are polyclonal in origin. The clonal rearrangements of Ig and / or TCR genes suggest that monoclonal clonal hyperplasia has the tendency of malignant transformation and its potential, which may help to predict the prognosis.