A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis
A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis
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DOI：
10.1182/blood-2014-04-570010
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发表时间：
2014-10-09
期刊：
影响因子：
20.3
通讯作者：
Schoenland, Stefan
中科院分区：
文献类型：
--
作者：
Palladini, Giovanni;Hegenbart, Ute;Schoenland, Stefan
70%的免疫球蛋白轻链（AL）淀粉样变性患者累及肾脏，但对肾脏受累的进展或可逆性知之甚少，肾脏缓解的标准从未得到验证。纳入了帕维亚（n = 461，试验队列）和海德堡（n = 271，验证队列）中心的新诊断患者。蛋白尿>5 g/24 h和估计肾小球滤过率（eGFR）降低≥ 25%预测两个队列中肾存活率较差，并被用作肾进展的标准。蛋白尿减少>= 30%或低于0.5 g/24 h而无肾脏进展是肾脏缓解的标准，与测试和验证人群中较长的肾脏生存期相关。6个月时血液学非常好的部分或完全缓解改善了两个人群的肾脏结局。我们确定并验证了肾脏受累的分期系统以及早期评估AL淀粉样变性肾脏反应和进展的标准，这些标准应用于临床实践和试验设计。
The kidney is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or reversibility of renal involvement, and criteria for renal response have never been validated. Newly diagnosed patients from the Pavia (n = 461, testing cohort) and Heidelberg (n = 271, validation cohort) centers were included. Proteinuria >5 g/24h and estimated glomerular filtration rate (eGFR) = 25% eGFR decrease predicted poor renal survival in both cohorts and was adopted as criterion for renal progression. A decrease in proteinuria by >= 30% or below 0.5 g/24 h without renal progression was the criterion for renal response, being associated with longer renal survival in the testing and validation populations. Hematologic very good partial or complete remission at 6 months improved renal outcome in both populations. We identified and validated a staging system for renal involvement and criteria for early assessment of renal response and progression in AL amyloidosis that should be used in clinical practice and trial design.