肾病综合征揭示恶性胸腺瘤
[Nephrotic syndrome revealing malignant thymoma].
作者信息
Schillinger F, Milcent T, Wolf C, Gulino R, Montagnac R
机构信息
Services de Néphrologie et d'Hémodialyse des Centres Hospitaliers de Troyes.
出版信息
Presse Med. 1998 Jan 17;27(2):60-3.
BACKGROUND
A well-established manifestation of neoplastic disease, nephrotic syndrome is infrequently associated with thymoma. Only 18 cases have been reported in the literature.
CASE REPORTS
A 65-year-old man and a 60-year-old woman were seen for nephrotic syndrome. Minimal change renal disease was observed in the first patient whose nephrotic syndrome was steroid resistant. The second patient had membranous glomerulopathy and pure red cell aplasia. In both cases, nephrotic syndrome revealed thymoma.
DISCUSSION
The histological lesions in 17 of the 18 biopsied cases reported in the literature were minimal change in 10, focal segmental glomerulonephritis in 4, proliferative glomerulonephritis in 2, and membranous glomerulopathy in only one. The outcome of the nephrotic syndrome was dependent on the success of the thymoma treatment. Some patients responded to steroid and immunosuppressive agents. Pure red cell aplasia is uncommon and prognosis is poor. It can be successfully treated with cyclosporin A as in our second case.
背景
肾病综合征是肿瘤性疾病的一种公认表现，与胸腺瘤的关联并不常见。文献中仅报道了18例。
病例报告
一名65岁男性和一名60岁女性因肾病综合征前来就诊。首例患者为微小病变型肾病，其肾病综合征对类固醇耐药。第二例患者患有膜性肾小球病和纯红细胞再生障碍。在这两例中，肾病综合征均发现合并胸腺瘤。
讨论
文献报道的18例活检病例中，17例的组织学病变为：10例为微小病变，4例为局灶节段性肾小球肾炎，2例为增殖性肾小球肾炎，仅1例为膜性肾小球病。肾病综合征的转归取决于胸腺瘤治疗的成效。部分患者对类固醇和免疫抑制剂有反应。纯红细胞再生障碍并不常见，预后较差。如我们的第二例患者，可用环孢素A成功治疗。
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