Angioimmunoblastic T-cell lymphoma and membranous nephropathy: a still unreported association
Angioimmunoblastic T-cell lymphoma and membranous nephropathy: a still unreported association
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DOI：
10.1007/s10157-010-0266-3
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发表时间：
2010-06-01
影响因子：
2.3
通讯作者：
Sawada, Ken-ichi
中科院分区：
文献类型：
--
作者：
Togashi, Masaru;Wakui, Hideki;Sawada, Ken-ichi
1例21岁男性淋巴结病合并coombs阳性溶血性贫血接受类固醇维持治疗。并发肾病综合征，淋巴结肿大。淋巴结检查发现血管免疫母细胞t细胞淋巴瘤（AITL）。肾活检标本光镜显示膜性肾病（MN）的典型特征，如冒泡外观和刺状形成。免疫荧光研究未发现明显的免疫球蛋白沉积。电镜显示肾小球基底膜上皮侧有稀疏的退行性物质，其间有尖刺。这些独特的组织学结果提示继发性MN。大剂量类固醇治疗，再加上六个疗程的环磷酰胺、阿霉素、长春新碱和强的松龙（CHOP）治疗，改善了他的症状。随访1年，患者健康状况良好，无复发迹象。肾小球表现很少与AITL相关。据我们所知，这是第一例因MN与AITL相关的肾病综合征的报道。
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.