Nishi J, Maruyama I
Department of Pediatrics, Faculty of Medicine, Kagoshima University, Japan.
Leuk Lymphoma. 2000 Jul;38(3-4):387-94. doi: 10.3109/10428190009087030.
Castleman's disease is a lymphoproliferative disorder of unknown etiology characterized by enlarged hyperplastic lymph nodes with marked vascular proliferation. To evaluate the possible involvement of vascular endothelial growth factor (VEGF) in the pathogenesis of Castleman's disease, we studied VEGF expression in sera and lymph nodes from four patients with either the plasma-cell type or mixed type of Castleman's disease. Clinically, one patient had the multicentric type and the others the localized type. The VEGF levels of the sera and the supernatants of the cultured lymph nodes were higher than those of normal controls. VEGF was strongly expressed in plasma cells in the interfollicular region of the lymph nodes, but rarely in normal lymph nodes. The disregulated IL-6 gene expression is considered to be a primary event that could be related to the etiology of this disease. Recently, Kaposi's sarcoma virus/human herpes virus 8 (KSHV/HHV-8) has been reported to be associated with a subset of the multicentric type of Castleman's disease, and a viral homologue of IL-6 (vIL-6) encoded by KSHV/HHV-8 has been shown to induce VEGF expression. Human IL-6 produced in the affected lymph nodes of Castleman's disease may induce paracrine VEGF-production by plasma cells and vascular proliferation in the lymph node. The confirmation of the role of VEGF in the pathogenesis of Castleman's disease may provide a therapeutic strategy.
卡斯特尔曼病是一种病因不明的淋巴增生性疾病，其特征为增生性淋巴结肿大并伴有显著的血管增生。为评估血管内皮生长因子（VEGF）在卡斯特尔曼病发病机制中的可能作用，我们研究了4例浆细胞型或混合型卡斯特尔曼病患者血清和淋巴结中的VEGF表达。临床上，1例为多中心型，其他为局限型。血清和培养淋巴结上清液中的VEGF水平高于正常对照组。VEGF在淋巴结滤泡间区的浆细胞中强烈表达，但在正常淋巴结中很少表达。IL-6基因表达失调被认为是可能与该病病因相关的主要事件。最近，有报道称卡波西肉瘤病毒/人类疱疹病毒8型（KSHV/HHV-8）与多中心型卡斯特尔曼病的一个亚型相关，并且KSHV/HHV-8编码的IL-6病毒同源物（vIL-6）已被证明可诱导VEGF表达。卡斯特尔曼病受累淋巴结中产生的人IL-6可能通过浆细胞诱导旁分泌VEGF生成以及淋巴结中的血管增生。证实VEGF在卡斯特尔曼病发病机制中的作用可能会提供一种治疗策略。
