Miura Naoto, Suzuki Keisuke, Yoshino Masabumi, Kitagawa Wataru, Yamada Harutaka, Ohtani Hiroshi, Joh Kensuke, Imai Hirokazu
Department of Internal Medicine, Division of Nephrology and Rheumatology, Aichi Medical University School of Medicine, Aichi, Japan.
Am J Kidney Dis. 2006 Jul;48(1):e3-9. doi: 10.1053/j.ajkd.2006.03.084.
A 70-year-old man with angioimmunoblastic T-cell lymphoma developed acute renal failure. Laboratory data showed decreased levels of serum C3, C4, and CH50, elevated immunoglobulin M (IgM) levels, and the presence of cryoglobulinemia (IgM-lambda). Renal biopsy showed membranoproliferative glomerulonephritis-like lesions with azan-red-stained thrombi in the glomerular capillary lumen. Immunofluorescence showed that IgM-lambda stained strongly in the glomerular capillary lumen, equal to the azan-red-stained thrombi, whereas C3 and C4 staining was negative. Electron microscopy showed electron-dense deposits in the subendothelial space and glomerular thrombi lacking fine fibrillar structure. These findings suggest that cryoglobulin, which consists of monoclonal IgM-lambda, induced glomerular thrombi and acute renal failure in a patient with angioimmunoblastic T-cell lymphoma.
一名患有血管免疫母细胞性T细胞淋巴瘤的70岁男性发生了急性肾衰竭。实验室数据显示血清C3、C4和CH50水平降低,免疫球蛋白M(IgM)水平升高,且存在冷球蛋白血症(IgM-λ)。肾活检显示膜增生性肾小球肾炎样病变,肾小球毛细血管腔内有偶氮红染色的血栓。免疫荧光显示IgM-λ在肾小球毛细血管腔内染色强烈,与偶氮红染色的血栓相同,而C3和C4染色为阴性。电子显微镜显示内皮下间隙有电子致密沉积物,且肾小球血栓缺乏细纤维结构。这些发现表明,由单克隆IgM-λ组成的冷球蛋白在一名血管免疫母细胞性T细胞淋巴瘤患者中诱导了肾小球血栓形成和急性肾衰竭。