Kurien A A, Fernando M E
Department of Pathology, Renopath Center for Renal and Urological Pathology, Chennai, Tamil Nadu, India.
Department of Nephrology, Government Stanley Medical College, Chennai, Tamil Nadu, India.
Indian J Nephrol. 2018 Mar-Apr;28(2):160-163. doi: 10.4103/ijn.IJN_297_16.
Patients with multiple myeloma (MM) often develop renal manifestations. The majority of cases present as cast nephropathy, amyloid light-chain (AL) amyloidosis, and monoclonal immunoglobulin deposition disease. AL amyloidosis usually involves the glomeruli, blood vessels, and interstitium. It is extremely uncommon to find isolated intratubular deposition of AL amyloid. Our patient presented with rapid worsening of renal function due to isolated intratubular deposition of AL amyloid, where the biopsy revealed amyloid proximal tubulopathy and amyloid cast nephropathy. Our case provides new insights into the complicated pathophysiology of the abnormal light chains in MM. This case is, to our knowledge, the second case of amyloid proximal tubulopathy reported in literature.
多发性骨髓瘤(MM)患者常出现肾脏表现。大多数病例表现为管型肾病、淀粉样轻链(AL)淀粉样变性和单克隆免疫球蛋白沉积病。AL淀粉样变性通常累及肾小球、血管和间质。孤立的肾小管内AL淀粉样蛋白沉积极为罕见。我们的患者因孤立的肾小管内AL淀粉样蛋白沉积导致肾功能迅速恶化,活检显示为淀粉样近端肾小管病和淀粉样管型肾病。我们的病例为MM中异常轻链复杂的病理生理学提供了新的见解。据我们所知,该病例是文献报道的第二例淀粉样近端肾小管病。