Histopathological improvement in kidney AL amyloid deposits following daratumumab-based chemotherapy and autologous peripheral blood stem cell transplantation: a case report.

IF 1 Q4 UROLOGY & NEPHROLOGY
Shuma Hirashio, Sayuri Ohnari, Moe Kadono, Takeshi Shimomura, Yoshiro Tachiyama, Takao Masaki
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Abstract

AL amyloidosis is characterized by extracellular deposition of immunoglobulin light-chain fibrils, frequently leading to kidney involvement and progressive organ dysfunction. We report a case of kidney AL amyloidosis secondary to multiple myeloma in which daratumumab-based chemotherapy combined with autologous peripheral blood stem cell transplantation resulted in marked improvement, as confirmed by sequential kidney biopsy. The initial biopsy revealed vascular-dominant amyloid deposition, whereas the second biopsy obtained 2 years later demonstrated a marked reduction in amyloid burden, particularly within the vascular compartments. This histopathological improvement corresponded with hematologic remission and stabilization of kidney function. The observed reduction in tissue amyloid deposits was presumed to result from suppression of amyloidogenic light chains, leading to inhibition of new fibril formation and subsequent tissue remodeling. Our findings provide direct pathological evidence that daratumumab-based combination therapy, through immune-mediated plasma cell depletion, may indirectly promote the regression of amyloid deposits and contribute to functional recovery.

达拉图单抗化疗和自体外周血干细胞移植后肾AL淀粉样蛋白沉积的组织病理学改善:一例报告。
AL淀粉样变的特征是免疫球蛋白轻链原纤维的细胞外沉积,经常导致肾脏受累和进行性器官功能障碍。我们报告一例继发于多发性骨髓瘤的肾AL淀粉样变性患者,经连续肾活检证实,以达拉图单抗为基础的化疗联合自体外周血干细胞移植显著改善。最初的活检显示以血管为主的淀粉样蛋白沉积,而2年后的第二次活检显示淀粉样蛋白负荷明显减少,特别是在血管室内。这种组织病理学上的改善与血液学缓解和肾功能稳定相一致。观察到的组织淀粉样蛋白沉积的减少被认为是由于淀粉样蛋白轻链的抑制,导致新纤维形成和随后的组织重塑的抑制。我们的研究结果提供了直接的病理证据,表明以达拉图单抗为基础的联合治疗,通过免疫介导的浆细胞耗竭,可能间接促进淀粉样蛋白沉积的消退,并有助于功能恢复。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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