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卡斯特曼病:一所大学医院中一种罕见淋巴增生性疾病的研究

Castleman's disease: a study of a rare lymphoproliferative disorder in a university hospital.

作者信息

Al-Amri A M, Abdul-Rahman I S, Ghallab K Q

机构信息

King Fahd University Hospital, Al-Khobar, Saudi Arabia.

出版信息

Gulf J Oncolog. 2010 Jan(7):25-36. doi: 10.4137/cmbd.s2161.

Abstract

Castleman's disease (CD) is a group of rare lymphoproliferative disorders sharing characteristic clinical and histological features, and usually accompanied by a marked systemic inflammatory response. Two histological patterns of lymph nodes were described: the hyaline-vascular and plasma-cell types. The former is more common (80-90%) and tends to be localized. The plasma cell type is more aggressive and usually multicentric. It is interesting that the inflammatory manifestations seem to be related to a lymph node lesion, because the systemic symptoms and inflammatory activity can return to normal after surgical excision or successful medical treatment of the disease. We report here our 15-year experience with this rare disease in King Fahd Hospital of the University, Al-Khobar, Saudi Arabia, focusing on the clinical features, therapy, and patients'outcome.

摘要

卡斯特曼病(CD)是一组罕见的淋巴增殖性疾病,具有特征性的临床和组织学特征,通常伴有明显的全身炎症反应。淋巴结有两种组织学类型:透明血管型和浆细胞型。前者更为常见(80%-90%),且往往为局限性。浆细胞型更具侵袭性,通常为多中心性。有趣的是,炎症表现似乎与淋巴结病变有关,因为在手术切除或成功的药物治疗该疾病后,全身症状和炎症活动可恢复正常。我们在此报告我们在沙特阿拉伯胡拜尔法赫德国王大学医院对这种罕见疾病15年的经验,重点关注临床特征、治疗及患者结局。

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