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Castleman 病的肾脏受累。

Renal involvement in Castleman disease.

机构信息

Department of Pathology, Hopital Europeen Georges Pompidou, AP-HP-Universite Paris Descartes, Paris, France.

出版信息

Nephrol Dial Transplant. 2011 Feb;26(2):599-609. doi: 10.1093/ndt/gfq427. Epub 2010 Jul 23.

DOI:10.1093/ndt/gfq427
PMID:20656751
Abstract

BACKGROUND

Castleman disease (CD), or angiofollicular lymph-node hyperplasia, is an atypical lymphoproliferative disorder with heterogeneous clinical manifestations. Renal involvement in CD has been described in only single-case reports, which have included various types of renal diseases.

METHODS

Nineteen patients with histologically documented CD and renal biopsies available were included. Clinical features and renal histological findings were reviewed, and the available samples were immunolabelled with anti-vascular endothelial growth factor (VEGF) antibody.

RESULTS

Nineteen CD cases were identified: 89% were multicentric, and 84% were of the plasma-cell or mixed type. Four cases (21%) were associated with human immunodeficiency virus (HIV) infection. Among HIV-negative patients, two main patterns of renal involvement were found: (i) a small-vessel lesions group (SVL) (60%) with endotheliosis and glomerular double contours in all patients and with superimposed glomerular/arteriolar thrombi or mesangiolysis in most; and (ii) AA amyloidosis (20%). Renal histology was more heterogeneous among HIV-positive patients. Decreases in glomerular VEGF were observed only in some patients with SVL, whereas VEGF staining was normal in all other histological groups. Interestingly, glomerular VEGF loss associated with SVL was correlated with plasma C-reactive protein levels, a marker of CD activity.

CONCLUSIONS

Small-vessel lesions are the most frequent renal involvement in CD, whereas loss of glomerular VEGF is correlated with CD activity and could have a role in SVL pathophysiology.

摘要

背景

Castleman 病(CD),又称血管滤泡性淋巴组织增生,是一种临床表现多样的非典型淋巴组织增生性疾病。CD 肾脏受累仅在个案报道中描述过,包括各种类型的肾脏疾病。

方法

纳入了 19 例经组织学证实的 CD 患者,这些患者均有肾活检样本。对这些患者的临床特征和肾脏组织学发现进行了回顾,并用抗血管内皮生长因子(VEGF)抗体对可用样本进行免疫标记。

结果

共确定了 19 例 CD 病例:89%为多中心性,84%为浆细胞型或混合细胞型。4 例(21%)与人类免疫缺陷病毒(HIV)感染相关。在 HIV 阴性患者中,发现了两种主要的肾脏受累模式:(i)小血管病变组(SVL)(60%),所有患者均有内皮细胞增多和肾小球双轮廓,大多数患者伴有肾小球/小动脉血栓形成或系膜溶解;(ii)AA 淀粉样变性(20%)。在 HIV 阳性患者中,肾脏组织学更具异质性。仅在一些 SVL 患者中观察到肾小球 VEGF 减少,而所有其他组织学组的 VEGF 染色均正常。有趣的是,与 SVL 相关的肾小球 VEGF 丢失与 CD 活性相关,并且可能在 SVL 病理生理学中发挥作用。

结论

小血管病变是 CD 最常见的肾脏受累,而肾小球 VEGF 的丢失与 CD 活性相关,并可能在 SVL 病理生理学中发挥作用。

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