原发性局限性下尿路淀粉样变

PRIMARY LOCALIZED AMYLOIDOSIS OF THE LOWER URINARY TRACT

目的 :复习文献并结合我院诊治病例 ,探讨原发性局限性下尿路淀粉样变的发病与分类 ,诊断和治疗。方法 :采用膀胱部分切除术及术后DMSO膀胱内灌注治疗原发性局限性膀胱淀粉样变 ,病变段尿道切除加尿路重建治疗原发性局限性尿道淀粉样变。结果 :所有病例经高锰酸钾氧化后刚果红复染反应而确诊 ,治疗后随访 6年未见复发。结论 :原发性局限性下尿路淀粉样变的诊断主要依赖于病理学检查和刚果红复染反应 ;DMSO膀胱内灌注可作为保留膀胱的原发性局限性膀胱淀粉样变的治疗方法 ,而病变段尿道切除加尿路重建对治疗局限性尿道淀粉样变效果肯定。

Objective:To determine the diagnosis and treatment of primary localized amyloidosis of the lower urinary tract by presenting our cases and reviewing the published papers.Methods:Two cases were diagnosed as primary localized amyloidosis located in the bladder and urethra separately by using pathological analysis. The bladder case was treated with partial cystectomy plus intravesical DMSO,and another with complete removal of the mass and penile urethral.Results:Two cases who were followed up on stablized for six years.Conclusions:The pathological analysis with Congo-red staining is the key evidence for the diagnosis of Primary localized amyloidosis of the lower urinary tract. The surgical therapy is useful for the treatment in urethral case,whereas intravesical DMSO can be an option of bladder saving measure in the bladder case.