Digital whole-slide imaging of changes in amyloid after peripheral blood stem cell transplantation in patients with amyloid light-chain amyloidosis.

IF 2.5 4区医学 Q2 PATHOLOGY Pathology International Pub Date : 2024-07-17 DOI:10.1111/pin.13466

Kei Kono, Naoki Sawa, Atsushi Wake, Yukako Shintani-Domoto, Takeshi Fujii, Yutaka Takazawa, Yoshifumi Ubara, Kenichi Ohashi

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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.

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淀粉样轻链淀粉样变性病患者外周血干细胞移植后淀粉样蛋白变化的数字全切片成像。

外周血干细胞移植（PBSCT）使淀粉样轻链（AL）淀粉样变性病变得可以治疗。PBSCT后，可实现血液学完全缓解（HCR），从而改善肾脏预后。本研究旨在评估活检样本的全切片成像是否显示AL淀粉样变性患者治疗后淀粉样沉积物减少。患者分为三组：A组（n = 8），不符合PBSCT条件，接受其他疗法治疗；B组（n = 11），接受PBSCT治疗并获得HCR；C组（n = 5），接受PBSCT治疗但未获得HCR。采用数字全切片成像技术比较了治疗前后的临床表现以及肾小球、间质和血管中的淀粉样蛋白沉积情况。与其他组相比，B 组的蛋白尿和低白蛋白血症得到了改善，B 组的肾小球淀粉样蛋白沉积改善程度高于肾间质和血管。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组织学预后。即使在临床HCR后，肾组织中淀粉样蛋白的消失也可能需要很长时间。

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来源期刊

Pathology International 医学-病理学

CiteScore

4.50

自引率

4.50%

发文量

102

审稿时长

12 months

期刊介绍： Pathology International is the official English journal of the Japanese Society of Pathology, publishing articles of excellence in human and experimental pathology. The Journal focuses on the morphological study of the disease process and/or mechanisms. For human pathology, morphological investigation receives priority but manuscripts describing the result of any ancillary methods (cellular, chemical, immunological and molecular biological) that complement the morphology are accepted. Manuscript on experimental pathology that approach pathologenesis or mechanisms of disease processes are expected to report on the data obtained from models using cellular, biochemical, molecular biological, animal, immunological or other methods in conjunction with morphology. Manuscripts that report data on laboratory medicine (clinical pathology) without significant morphological contribution are not accepted.