Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55902, USA.
Am J Kidney Dis. 2010 Nov;56(5):971-6. doi: 10.1053/j.ajkd.2010.06.018. Epub 2010 Sep 25.
Plasma cell dyscrasias can present as myeloma cast nephropathy, AL amyloid, or light chain deposition disease. We describe the unusual kidney biopsy findings of concurrent myeloma cast nephropathy and glomerular nonamyloid fibrillary deposits composed of immunoglobulin G (IgG) heavy chains and λ light chains in a patient with multiple myeloma who presented with acute renal failure. We performed laser microdissection and mass spectrometry-based proteomic analysis, which showed that the fibrillary deposits likely contained Igγ1 constant region and λ light chain constant region, whereas κ light chains and serum amyloid P proteins were absent. Treatment of multiple myeloma resulted in resolution of the renal manifestations, suggesting a common underlying mechanism for the cast nephropathy and heavy and light chain deposition disease. We show that laser microdissection and mass spectrometry is an extremely useful ancillary test for the diagnosis of heavy and light chain deposition diseases.
浆细胞异常可表现为骨髓瘤管型肾病、AL 淀粉样变性或轻链沉积病。我们描述了一位多发性骨髓瘤患者的不常见肾脏活检结果,该患者表现为急性肾衰竭,其肾脏活检同时存在骨髓瘤管型肾病和由 IgG 重链和 λ 轻链组成的肾小球非淀粉样纤维状沉积物。我们进行了激光显微切割和基于质谱的蛋白质组学分析,结果表明纤维状沉积物可能含有 Igγ1 恒定区和 λ 轻链恒定区,而 κ 轻链和血清淀粉样蛋白 P 蛋白缺失。多发性骨髓瘤的治疗导致肾脏表现得到缓解,提示管型肾病和重链和轻链沉积病具有共同的潜在机制。我们表明,激光显微切割和质谱分析是诊断重链和轻链沉积病的一种极其有用的辅助检测方法。