器官功能障碍
淀粉样变性
医学
淀粉样变性
肾
器官系统
内科学
胃肠病学
疾病
病理
免疫学
免疫球蛋白轻链
抗体
败血症
作者
Andrew Staron,Lisa Mendelson,Tracy Joshi,Natasha J Burke,Vaishali Sanchorawala
摘要
Summary Patients 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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