全身性 AL 淀粉样变性患者虽有深度血液学反应,但器官恢复仍受阻的因素

IF 5.1 2区 医学 Q1 HEMATOLOGY British Journal of Haematology Pub Date : 2024-09-14 DOI:10.1111/bjh.19766
Andrew Staron, Lisa M. Mendelson, Tracy Joshi, Natasha Burke, Vaishali Sanchorawala
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引用次数: 0

摘要

摘要AL淀粉样变性患者尽管获得了血液学完全应答(hemCR),但仍可能出现持续的器官功能障碍。我们旨在确定143名实现血液学完全应答≥6个月的患者中器官无应答的因素。肾脏、心脏和肝脏无应答的患者分别为 40/117(34%)、19/68(28%)和 3/17(18%)。不同器官系统的诱发因素各不相同。肾脏无应答者在确诊时器官功能障碍的程度更严重,而心脏无应答者的λ型淀粉样蛋白生成轻链则多得不成比例。大多数无明显器官无应答原因的患者都有可检测到的残留克隆疾病。阻碍AL淀粉样变性器官恢复的因素错综复杂。
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Factors impeding organ recovery despite a deep haematological response in patients with systemic AL amyloidosis
SummaryPatients with AL amyloidosis can have persistent organ dysfunction despite achieving a haematological complete response (hemCR). We aimed to identify factors for organ non‐response among 143 patients who achieved hemCR for ≥6 months. Kidney, heart and liver non‐response were observed in 40/117 (34%), 19/68 (28%) and 3/17 (18%) patients respectively. Predisposing factors varied by organ system. Kidney non‐responders had more advanced organ dysfunction at diagnosis, whereas heart non‐responders had disproportionately more lambda‐typic amyloidogenic light chains. Most patients without an apparent reason for organ non‐response had detectable residual clonal disease. The interplay of factors impeding organ recovery in AL amyloidosis is complex.
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来源期刊
CiteScore
8.60
自引率
4.60%
发文量
565
审稿时长
1 months
期刊介绍: The British Journal of Haematology publishes original research papers in clinical, laboratory and experimental haematology. The Journal also features annotations, reviews, short reports, images in haematology and Letters to the Editor.
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