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一名患有血管免疫母细胞性T细胞淋巴瘤和增殖性肾小球肾炎的男性。

A male with angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis.

作者信息

De Samblanx Hadewijch, Verhoef Gregor, Zachée Pierre, Vandenberghe Peter

机构信息

Hematology Unit, University Hospital Leuven, Leuven, Belgium.

出版信息

Ann Hematol. 2004 Jul;83(7):455-9. doi: 10.1007/s00277-003-0828-5. Epub 2004 Mar 18.

Abstract

In this article we present the case report of a 67-year-old male with a nephrotic syndrome due to a proliferative glomerulonephritis, associated with an angioimmunoblastic T-cell lymphoma. Diagnosis was made on an axillary lymph node biopsy and showed expanded T-cell areas with multiple blood vessels, small mature lymphocytes, eosinophils, and plasma cells. A kidney biopsy was suggestive for a proliferative glomerulonephritis with intra- and extracapillary proliferation. Hypercellular glomeruli were seen, as well as multiple floride crescents. Interstitial edema and fibrosis were absent. Immunohistochemical reactions were negative; there was some mesangial reaction with IgM in the glomeruli. Treatment with high-dose corticosteroids was initiated, with clinical improvement, and was immediately followed by therapy with cyclophosphamide, hydroxydaunomycin, vincristine, and prednisone (CHOP), which induced complete remission with a follow-up of 1 year. To our knowledge, the association of angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis has only been described twice. It concerned elderly men who developed acute renal failure a couple of months after the diagnosis of an angioimmunoblastic T-cell lymphoma. In both, immunoglobulin-containing dense deposits within glomeruli were observed, which was not the case in our patient, where only some mesangial colorization of the IgM in the glomeruli was seen.

摘要

在本文中,我们报告了一例67岁男性患者,其因增殖性肾小球肾炎导致肾病综合征,并伴有血管免疫母细胞性T细胞淋巴瘤。诊断通过腋窝淋巴结活检做出,结果显示T细胞区域扩大,伴有多条血管、小成熟淋巴细胞、嗜酸性粒细胞和浆细胞。肾活检提示为伴有毛细血管内和毛细血管外增殖的增殖性肾小球肾炎。可见肾小球细胞增多,以及多个明显的新月体。无间质水肿和纤维化。免疫组化反应为阴性;肾小球内有一些IgM的系膜反应。开始使用高剂量皮质类固醇治疗,临床症状改善,随后立即采用环磷酰胺、羟基柔红霉素、长春新碱和泼尼松(CHOP)治疗,随访1年诱导完全缓解。据我们所知,血管免疫母细胞性T细胞淋巴瘤与增殖性肾小球肾炎的关联仅被描述过两次。涉及的是在诊断血管免疫母细胞性T细胞淋巴瘤几个月后出现急性肾衰竭的老年男性。在这两例中,肾小球内均观察到含免疫球蛋白的致密沉积物,而在我们的患者中并非如此,仅在肾小球内可见一些IgM的系膜着色。

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