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血管免疫母细胞性 T 细胞淋巴瘤和膜性肾病:一种仍未报道的关联。

Angioimmunoblastic T-cell lymphoma and membranous nephropathy: a still unreported association.

机构信息

Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Akita, Akita, 010-8543, Japan.

出版信息

Clin Exp Nephrol. 2010 Jun;14(3):288-93. doi: 10.1007/s10157-010-0266-3. Epub 2010 Feb 23.

Abstract

A 21-year-old man with lymphadenopathy and Coombs-positive hemolytic anemia had been treated with steroid maintenance therapy. He developed nephrotic syndrome with size increase of lymphadenopathy. Lymph node examination disclosed angioimmunoblastic T-cell lymphoma (AITL). Light microscopy of a renal biopsy specimen showed typical features of membranous nephropathy (MN), such as bubbling appearance and spike formation. Immunofluorescence studies revealed no significant deposition of immunoglobulins. Electron microscopy showed sparse degenerative materials on the epithelial side of the glomerular basement membranes, with intervening spikes. These unique histological findings suggested secondary MN. High-dose steroid therapy followed by six courses of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) therapy improved his symptoms. One-year follow-up revealed the patient in good health without any signs of relapse. Glomerular manifestations have rarely been reported in association with AITL. To our knowledge, this is the first reported case of nephrotic syndrome due to MN associated with AITL.

摘要

一位 21 岁的男性因淋巴结病和抗 Coombs 阳性溶血性贫血而接受了类固醇维持治疗。他出现了肾病综合征并伴有淋巴结病增大。淋巴结检查显示为血管免疫母细胞性 T 细胞淋巴瘤(AITL)。肾活检标本的光镜检查显示出典型的膜性肾病(MN)特征,如冒泡外观和刺形成。免疫荧光研究显示免疫球蛋白无明显沉积。电子显微镜显示肾小球基底膜上皮侧有稀疏的退行性物质,其间有刺。这些独特的组织学发现提示为继发性 MN。大剂量类固醇治疗后,再进行六轮环磷酰胺、多柔比星、长春新碱和泼尼松(CHOP)治疗,改善了他的症状。一年的随访显示患者身体健康,没有任何复发迹象。肾小球表现与 AITL 相关的情况很少有报道。据我们所知,这是首例因 MN 相关的 AITL 引起的肾病综合征病例报告。

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