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使用计算机断层扫描引导下肾活检诊断轻链沉积病

Light Chain Deposition Disease Diagnosed Using Computed Tomography-Guided Kidney Biopsy.

作者信息

Shimamura Yoshinosuke, Ogawa Yayoi, Takizawa Hideki, Hayashi Toshiaki, Sakurai Yasuo

机构信息

Department of Nephrology, Teine Keijinkai Medical Center, Sapporo, JPN.

Department of Renal Pathology, Hokkaido Renal Pathology Center, Sapporo, JPN.

出版信息

Cureus. 2021 May 18;13(5):e15102. doi: 10.7759/cureus.15102.

Abstract

Light chain deposition disease (LCDD) is characterized by the deposition of monoclonal immunoglobulin light chains in the kidney, which can cause end-stage kidney disease if not treated. While kidney biopsy is required for definitive diagnosis, choosing an appropriate biopsy method may be problematic when examining patients with atrophic kidneys. A 66-year-old Japanese man was referred to our institution with a three-month history of leg edema. Clinical investigations revealed proteinuria levels of 7.5 g/day. CT-guided percutaneous kidney biopsy was selected as the biopsy method because atrophic kidneys were poorly visualized on ultrasonography. Kidney biopsy revealed nodular glomerulosclerosis, exclusive deposition of the κ chain, and powdery electron-dense deposits, all of which were indicative of LCDD. Bence-Jones protein was detected in the urine. The patient also had an abnormal serum-free light chain ratio. Bone marrow biopsy revealed multiple myeloma; therefore, the patient was diagnosed to have LCDD with multiple myeloma. The patient was treated with daratumumab, bortezomib, cyclophosphamide, and dexamethasone. After a one-year follow-up, the patient had hematological and renal responses without any treatment-related adverse effects. Our case demonstrates the effectiveness of daratumumab as a treatment for LCDD with nephrotic-range proteinuria. Additionally, we suggest that CT-guided kidney biopsy should be considered as a diagnostic test in patients with kidney atrophy when making a definitive diagnosis.

摘要

轻链沉积病(LCDD)的特征是单克隆免疫球蛋白轻链在肾脏沉积,若不治疗可导致终末期肾病。虽然明确诊断需要进行肾活检,但在检查萎缩性肾脏患者时,选择合适的活检方法可能存在问题。一名66岁的日本男性因腿部水肿3个月被转诊至我院。临床检查发现蛋白尿水平为7.5g/天。由于超声检查显示萎缩性肾脏显像不佳,因此选择CT引导下经皮肾活检作为活检方法。肾活检显示结节性肾小球硬化、κ链的排他性沉积以及粉末状电子致密沉积物,所有这些均提示LCDD。尿中检测到本周蛋白。患者血清游离轻链比值也异常。骨髓活检显示多发性骨髓瘤;因此,该患者被诊断为合并多发性骨髓瘤的LCDD。患者接受了达雷妥尤单抗、硼替佐米、环磷酰胺和地塞米松治疗。经过一年的随访,患者出现血液学和肾脏反应,且无任何治疗相关不良反应。我们的病例证明了达雷妥尤单抗治疗合并肾病范围蛋白尿的LCDD的有效性。此外,我们建议在明确诊断时,对于肾脏萎缩的患者,应考虑将CT引导下肾活检作为一种诊断检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/16cd/8212893/e16a5675dd59/cureus-0013-00000015102-i01.jpg

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