局灶性Castleman病17例报告并文献复习

Local Castleman s Disease: A Report of 17 Cases witn Literature Review

背景与目的:Castleman病(Castleman;sdisease,CD)又称血管滤泡性淋巴组织增生或巨大淋巴结增生,是一种少见的原因未明的反应性淋巴结病。本研究报告17例局灶性Castleman病(local Castleman;s disease,LCD)的临床特点和疗效,结合复习相关文献,以提高对LCD的诊治水平。方法:回顾性分析中山大学肿瘤防治中心从1995年8月至2006年7月收治的17例LCD患者临床资料。结果:无临床症状患者14例,有临床症状者3例,淋巴结呈单个或多个聚集,淋巴结最大径1.2～10.4cm,其中11例位于颈部,3例位于纵隔,位于肺部、肠系膜、肾上腺区各1例。其中透明血管型15例,浆细胞型1例,混合型1例,均经术后病理确诊。1例透明血管型CD出现脾大、白蛋白降低(25.6g/L)、球蛋白升高(80.0g/L)、大便潜血(+);1例浆细胞型CD出现中度贫血(95.0g/L)、尿蛋白(+)、大便潜血(+);余15例患者实验室检查结果均在正常范围内。17例患者均行肿物切除术,术后失访2例,余15例随访1～129个月,中位随访时间25个月,现均生存,无肿瘤复发。结论:LCD主要表现为单一部位的淋巴结肿大,以透明血管型为主,多无临床症状和实验室检查异常结果。CT检查对诊断有一定帮助,但确诊仍靠病理。手术切除疗效好,术后可长期生存。

BACKGROUND ＆ OBJECTIVE： Castleman＇s disease （CD）, also named angiofollicular lymph node hyperplasia or giant lymph node hyperplasia, is an uncommon reactive lymphadenopathy with unknown causes. This study was to report clinical features and therapeutic outcomes of 17 cases of local Castleman＇s disease （LCD）, review literatures to improve the diagnosis and management of CD. METHODS： Clinical data of 17 LCD patients, treated in Cancer Center of Sun Yat-sen University from Aug. 1995 to Jul. 2006, were reviewed. RESULTS： There were 3 cases with and 14 cases without clinical symptoms. A single lymph node or aggregation of multiple lymph nodes was observed in a single location. The longest diameters of involved lymph nodes ranged from 1.2 to 10.4 cm. The lesions were located in the neck （11 cases）, mediastinum （3 cases）, lung （1 case）, mesentery （1 case）, adrenal region （1 case）. Fifteen cases were hyaline vascular type,1 case was plasma cell type and 1 case was mixed type; all were confirmed pathologically. One hyaline vascular type LCD patient had splenomegaly, hypoalbumin （25.6 g/L）, hyperglobulin （80.0 g/L） and positive fecal occult blood. One plasma cell type LCD patient had anemia （95.0 g/L）, positive urine protein and positive fecal occult blood. The other 15 patients had normal laboratory results. All ratients underwent complete surgical resection. The duration of follow-up ranged from 1 to 129 moths with a median of 25 months. Fifteen followed-up patients were all alive without recurrence. CONCLUSIONS： LCD patients mainly have lymphadenectasis in a single location, no clinical symptoms and normal laboratory results, with a majority of hyaline vascular type. Although CT is helpful for the diagnosis of LCD, the final diagnosis depends on pathologic examination. LCD patients can live long without recurrence after complete surgical resection of the tumor.