The Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama, Toyama, 930-0194, Japan.
The Third Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama, Toyama, 930-0194, Japan.
CEN Case Rep. 2021 Nov;10(4):527-536. doi: 10.1007/s13730-021-00602-0. Epub 2021 Apr 27.
We had a 72-year-old man with advanced gastric cancer, poorly differentiated adenocarcinoma, receiving chemotherapy with S-1 (tegafur, gimeracil, and oteracil potassium) plus oxaliplatin. Ascites developed despite remission of gastric cancer and metastasis. Given no malignant cells in ascites, leg edema, renal impairment, hypoalbuminemia, and massive proteinuria, we diagnosed as nephrotic syndrome with microscopic hematuria. Renal biopsy showed membranoproliferative glomerulonephritis with no deposition of immunoglobulins and complements. Of note, electronic microscopy found organized deposits with microtubular structures in the glomerular capillary lumens and subendothelial spaces. The liquid chromatography-tandem mass spectrometry method detected fibrinogen alpha chain, beta chain, gamma chain, and fibronectin, and we eventually diagnosed cryofibrinogen-associated glomerulonephritis. Cryofibrinogen was not detected in plasma. He was expired at 5 months following renal biopsy due to the progression of refractory nephrotic syndrome. In addition to the detailed assessment of specifically organized deposits, the analysis using liquid chromatography-tandem mass spectrometry method is useful to diagnose cryofibrinogen-associated glomerulonephritis. We should consider cryofibrinogen-associated glomerulonephritis as a differential diagnosis when the patients with malignancy showed abnormal urinalysis and renal impairment, though it is a rare disease.
我们治疗了一位 72 岁的男性晚期胃癌患者,分化差的腺癌,接受 S-1(替加氟、吉美嘧啶、奥替拉西钾)联合奥沙利铂化疗。尽管胃癌和转移得到缓解,但仍出现腹水。腹水无恶性细胞,存在下肢水肿、肾功能不全、低白蛋白血症和大量蛋白尿,我们诊断为伴有镜下血尿的肾病综合征。肾活检显示为膜增生性肾小球肾炎,无免疫球蛋白和补体沉积。值得注意的是,电子显微镜发现肾小球毛细血管腔和内皮下空间有组织化沉积物和微管状结构。液相色谱-串联质谱法检测到纤维蛋白原 alpha 链、beta 链、gamma 链和纤维连接蛋白,最终诊断为冷纤维蛋白原相关肾小球肾炎。血浆中未检测到冷纤维蛋白原。肾活检后 5 个月,因难治性肾病综合征进展,患者死亡。除了详细评估特定的组织化沉积物外,液相色谱-串联质谱法的分析有助于诊断冷纤维蛋白原相关肾小球肾炎。当恶性肿瘤患者出现异常尿液和肾功能不全时,应考虑冷纤维蛋白原相关肾小球肾炎作为鉴别诊断,尽管这是一种罕见疾病。