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伴有与血管免疫母细胞性淋巴结病样T细胞淋巴瘤相关的异常、有组织的圆柱形沉积物的增殖性肾小球肾炎。

Proliferative glomerulonephritis with unusual, organized, cylindrical deposits associated with angioimmunoblastic lymphadenopathy-like T-cell lymphoma.

作者信息

Duwaji M S, Shemin D G, Medeiros L J, Esparza A R

机构信息

Department of Pathology, Rhode Island Hospital, Providence 02903, USA.

出版信息

Arch Pathol Lab Med. 1995 Apr;119(4):377-80.

PMID:7726733
Abstract

We describe an elderly man who developed angioimmunoblastic lymphadenopathy-like T-cell lymphoma, followed by acute renal failure 2 months later. Renal biopsy revealed proliferative glomerulonephritis, which was characterized by enlarged glomeruli with increased cellularity, thickened capillaries, intracapillary inflammatory cells, focal necrosis, and fibrin extravasation. Immunofluorescence studies revealed capillary and mesangial deposits of IgG, IgM, IgA, Ig kappa, Ig lambda, and C3. Electron microscopy revealed unusual, organized, electron-dense deposits in the capillary walls and mesangium. The deposits occurred as accumulations of large rigid tubules or cylinders, which, in longitudinal section, were double-walled. In transverse section, the deposits were annular or horseshoe shaped and occasionally had a central filament. The morphologic characteristics of these deposits are different from those seen in cryoglobulinemia or fibrillary and immunotactoid glomerulopathies. The significance of these deposits is uncertain; they may represent a cryoglobulin or an abnormal serum protein related to angioimmunoblastic lymphadenopathy-like T-cell lymphoma. The findings in this case expand the morphologic spectrum of glomerular lesions that may be associated with malignant lymphoproliferative disorders and, particularly, angioimmunoblastic lymphadenopathy-like T-cell lymphoma.

摘要

我们描述了一名老年男性,他患血管免疫母细胞性淋巴结病样T细胞淋巴瘤,2个月后出现急性肾衰竭。肾活检显示为增殖性肾小球肾炎,其特征为肾小球增大、细胞增多、毛细血管壁增厚、毛细血管内炎性细胞浸润、局灶性坏死及纤维蛋白外渗。免疫荧光研究显示IgG、IgM、IgA、Igκ、Igλ和C3在毛细血管和系膜沉积。电子显微镜检查显示在毛细血管壁和系膜中有异常的、有组织的、电子致密沉积物。这些沉积物表现为大的刚性小管或圆柱体的聚集,在纵切面上为双壁。在横切面上,沉积物呈环形或马蹄形,偶尔有中央细丝。这些沉积物的形态学特征不同于冷球蛋白血症或纤维样和免疫触须样肾小球病中所见。这些沉积物的意义尚不确定;它们可能代表一种冷球蛋白或与血管免疫母细胞性淋巴结病样T细胞淋巴瘤相关的异常血清蛋白。该病例的发现扩展了可能与恶性淋巴增殖性疾病,特别是血管免疫母细胞性淋巴结病样T细胞淋巴瘤相关的肾小球病变的形态学谱。

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