Chang Kung-Chao, Wang Yu-Chu, Hung Liang-Yi, Huang Wan-Ting, Tsou Jen-Hui, M Jones Dan, Song Hsiang-Lin, Yeh Yu-Min, Kao Lin-Yuan, Medeiros L Jeffrey
Department of Pathology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan.
Institute of Bioinformatics and Biosignal Transduction, College of Bioscience and Biotechnology, National Cheng Kung University, Tainan, Taiwan.
Mod Pathol. 2014 Jun;27(6):823-31. doi: 10.1038/modpathol.2013.202. Epub 2013 Nov 8.
Hyaline vascular Castleman disease is traditionally regarded as a reactive hyperplastic process. Occasional cases, however, have been reported with cytogenetic anomalies bringing this concept into question. In this study, we used conventional and methylation-specific polymerase chain reaction methods to assess the human androgen receptor α (HUMARA) gene in 29 female patients with hyaline vascular Castleman disease and compared the results with three cases of plasma cell Castleman disease and 20 cases of age-matched lymphoid hyperplasia. We also assessed for immunoglobulin gene and T-cell receptor gene rearrangements, and conventional cytogenetic analysis was performed in three cases of hyaline vascular Castleman disease. In cases with informative results, conventional and methylation-specific human androgen receptor α gene analyses yielded a monoclonal pattern in 10 of 19 (53%) and 17 of 23 (74%) cases of hyaline vascular Castleman disease, respectively. A monoclonal pattern was also detected in three cases of plasma cell Castleman disease but not in cases of lymphoid hyperplasia. The frequency of monoclonality was higher for lesions >5 cm in size (100%) and for the stromal-rich variant (91%). Cytogenetic abnormalities in stromal cells were revealed in two cases of hyaline vascular Castleman disease and no cases showed monoclonal immunoglobulin or T-cell receptor gene rearrangements. Follow-up data showed persistent disease in 4 of 23 (17%) patients. We conclude that hyaline vascular Castleman disease is often a monoclonal proliferation, most likely of lymph node stromal cells.
透明血管型Castleman病传统上被视为一种反应性增生过程。然而，偶尔有病例报告存在细胞遗传学异常，这使这一概念受到质疑。在本研究中，我们使用常规和甲基化特异性聚合酶链反应方法评估了29例女性透明血管型Castleman病患者的人类雄激素受体α（HUMARA）基因，并将结果与3例浆细胞型Castleman病和20例年龄匹配的淋巴组织增生病例进行了比较。我们还评估了免疫球蛋白基因和T细胞受体基因重排，并对3例透明血管型Castleman病进行了常规细胞遗传学分析。在结果有信息价值的病例中，常规和甲基化特异性人类雄激素受体α基因分析分别在19例透明血管型Castleman病患者中的10例（53%）和23例中的17例（74%）产生了单克隆模式。在3例浆细胞型Castleman病中也检测到单克隆模式，但在淋巴组织增生病例中未检测到。病变大小>5 cm的病例（100%）和富含基质的变体病例（91%）中单克隆性的频率更高。在2例透明血管型Castleman病中发现了基质细胞的细胞遗传学异常，没有病例显示单克隆免疫球蛋白或T细胞受体基因重排。随访数据显示，23例患者中有4例（17%）疾病持续存在。我们得出结论，透明血管型Castleman病通常是一种单克隆增殖，最有可能是淋巴结基质细胞的增殖。
