Nephrology and Dialysis Unit-CMID, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hospital of Turin, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
Nephrology and Dialysis Unit-CMID, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hospital of Turin, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
Autoimmun Rev. 2020 Sep;19(9):102622. doi: 10.1016/j.autrev.2020.102622. Epub 2020 Jul 12.
Immunoglobulin light chain amyloidosis is a rare systemic disease caused by monoclonal light chains (LCs) depositing in tissue as insoluble fibrils resulting in irreversible damage of vital organs. The mechanisms involved in aggregation and deposition of LCs are not fully understood, but CD138/38 plasma cells are undoubtedly involved in monoclonal LC production. We are reporting favorable effects on AL amyloidosis patients with renal involvement using the anti-CD38 monoclonal antibody Daratumumab. We speculate that research for the near future should be devoted to design similar therapeutic approaches for other diseases attributable to a plasma cell dyscrasia.
免疫球蛋白轻链淀粉样变性是一种罕见的系统性疾病，由单克隆轻链 (LC) 沉积在组织中形成不溶性原纤维引起，导致重要器官的不可逆损伤。LC 聚集和沉积的机制尚未完全阐明，但 CD138/38 浆细胞无疑参与了单克隆 LC 的产生。我们报告了使用抗 CD38 单克隆抗体达鲁单抗对伴有肾受累的 AL 淀粉样变性患者的有益作用。我们推测，未来的研究应该致力于设计针对其他由浆细胞功能紊乱引起的疾病的类似治疗方法。
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