Nephrology Department, Sichuan Provincial People's Hospital, Chengdu, 610072, China.
Medical School, University of Electronic Science and Technology of China, Chengdu, 611731, China.
BMC Nephrol. 2022 Mar 31;23(1):127. doi: 10.1186/s12882-022-02752-4.
Published literatures on repeat renal biopsy of AL amyloidosis have basically reached a consensus that amyloid material deposit does not disappear or diminish after satisfactory hematologic response, regardless of renal response. However, the need of a repeat renal biopsy in such situation is still controversial.
Here we reported a case of histologically confirmed λ Type renal AL amyloidosis who had been classified as Stage I and low risk at initial diagnosis. The patient received a total of six courses of CyBorD chemotherapy. She had achieved complete hematologic remission after two courses of chemotherapy but consistently had large amount of proteinuria over 10 g/day during follow up. A repeat renal biopsy was performed nine months after the first one and indicated mild to moderate increase of amyloid deposits as well as significant glomerulosclerosis and interstitial lesions, suggesting a lack of histological renal improvement despite her satisfactory hematologic response.
This case indicated renal involvement in AL amyloidosis could progress after successful hematologic treatment, and supported the value of repeat renal biopsy in the evaluation of AL amyloidosis patients lacking renal response despite of complete hematologic remission.
已发表的关于 AL 淀粉样变性重复肾活检的文献基本达成共识,即无论肾脏反应如何,在满意的血液学反应后,淀粉样物质沉积不会消失或减少。然而,在这种情况下是否需要重复肾活检仍存在争议。
本文报道了 1 例经组织学证实的 λ 型肾 AL 淀粉样变性患者,该患者在初始诊断时被归类为Ⅰ期和低危。该患者共接受了 6 个疗程的 CyBorD 化疗。她在两个疗程的化疗后达到完全血液学缓解,但在随访期间持续出现大量蛋白尿(>10 g/天)。第一次肾活检后 9 个月进行了重复肾活检,结果显示淀粉样物质沉积轻度至中度增加,以及明显的肾小球硬化和间质病变,提示尽管血液学反应良好,但组织学肾脏改善不足。
本病例表明 AL 淀粉样变性患者在成功进行血液学治疗后肾脏受累仍可进展,支持在完全血液学缓解但缺乏肾脏反应的 AL 淀粉样变性患者中重复肾活检的价值。