Abstract：
Peripheral blood stem cell transplantation (PBSCT) has made amyloid light-chain (AL) amyloidosis treatable. After PBSCT, hematological complete remission (HCR) can be achieved, leading to improved renal prognosis. The purpose of this study was to evaluate whether whole slide imaging of biopsy samples shows a post-treatment reduction in amyloid deposits in patients with AL amyloidosis. Patients were divided into three groups: Group A (n = 8), not eligible for PBSCT and treated with other therapies; Group B (n = 11), treated with PBSCT and achieved HCR; and Group C (n = 5), treated with PBSCT but did not achieve HCR. Clinical findings and amyloid deposition in glomeruli, interstitium, and blood vessels were compared before and after treatment using digital whole-slide imaging. Proteinuria and hypoalbuminemia improved more in Group B than in the other groups, and in Group B, amyloid deposition improved more in the glomeruli than in the interstitium and blood vessels. The long-term renal and survival prognosis was better in Group B than in the other groups. PBSCT can be expected to improve long-term clinical and renal histological prognosis in patients with AL amyloidosis who achieve HCR. Amyloid disappearance from renal tissue may take a long time even after clinical HCR.
摘要：
外周血干细胞移植（PBSCT）使淀粉样蛋白轻链（AL）淀粉样变性得以治疗。在PBSCT之后，可以实现血液学完全缓解（HCR），改善肾脏预后。这项研究的目的是评估活检样本的整个载玻片成像是否显示AL淀粉样变性患者治疗后淀粉样蛋白沉积减少。患者分为三组：A组（n=8），不符合PBSCT条件并接受其他疗法治疗；B组（n=11），用PBSCT治疗并达到HCR；和C组(n=5)，用PBSCT治疗，但未达到HCR。临床发现和肾小球淀粉样蛋白沉积，间质,使用数字全载玻片成像比较治疗前后的血管。B组蛋白尿和低蛋白血症的改善程度高于其他组，在B组中，肾小球中的淀粉样蛋白沉积比间质和血管中的淀粉样蛋白沉积改善得更多。B组的长期肾脏和生存预后优于其他组。PBSCT有望改善达到HCR的AL淀粉样变性患者的长期临床和肾脏组织学预后。甚至在临床HCR之后,淀粉样蛋白从肾组织的消失可能需要很长时间。
