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拼凑AL淀粉样变性中器官恢复的复杂谜题。

Piecing together the intricate puzzle of organ recovery in AL amyloidosis.

作者信息

Nuvolone Mario, Palladini Giovanni

机构信息

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo, Pavia, Italy.

出版信息

Br J Haematol. 2024 Dec;205(6):2119-2121. doi: 10.1111/bjh.19810. Epub 2024 Oct 3.

Abstract

In AL amyloidosis, the factors hindering organ recovery despite deep haematological responses to anti-clonal therapy remain elusive. The study by Staron and colleagues identifies tissue-specific factors influencing the lack of organ response, including host-related characteristics, the properties of the amyloidogenic precursor, the extent of organ compromise at diagnosis and the dynamics and depth of haematological response. While this study begins to unravel the complex mechanisms underlying organ recovery in AL amyloidosis, it also raises critical questions that warrant further investigation through future collaborative research. Commentary on: Staron et al. Factors impeding organ recovery despite a deep haematological response in patients with systemic AL amyloidosis. Br J Haematol 2024; 205:2268-2272.

摘要

在AL淀粉样变性中,尽管对抗克隆治疗有深度血液学反应,但阻碍器官恢复的因素仍不清楚。Staron及其同事的研究确定了影响器官无反应的组织特异性因素,包括宿主相关特征、淀粉样蛋白生成前体的特性、诊断时器官受损的程度以及血液学反应的动态变化和深度。虽然这项研究开始揭示AL淀粉样变性中器官恢复的复杂机制,但它也提出了关键问题,需要通过未来的合作研究进行进一步调查。评论文章:Staron等人。系统性AL淀粉样变性患者尽管有深度血液学反应但仍阻碍器官恢复的因素。《英国血液学杂志》2024年;205:2268 - 2272。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0b7/11637724/723320d55e0c/BJH-205-2119-g001.jpg

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