Department of Nephrology, Dialysis and Renal Transplantation, University Hospital of Poitiers, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Poitiers, France.
Department of Pathology, University Hospital of Poitiers, Poitiers, France.
Nephrol Dial Transplant. 2016 Jan;31(1):64-72. doi: 10.1093/ndt/gfv283. Epub 2015 Aug 19.
Light chain myeloma cast nephropathy (MCN) is the major cause of renal failure in multiple myeloma and strongly impacts patient survival. The role of kidney biopsy in the management of MCN is unclear.
Renal pathological findings were retrospectively studied in 70 patients with multiple myeloma and MCN. Patients were categorized according to the achievement or not of renal response, as defined by estimated glomerular filtration rate (eGFR) ≥ 30 mL/min/1.73 m(2) and/or dialysis independence at 3 months.
Thirty-two patients (46%) achieved a renal response. In the whole study population, the following parameters differed significantly between patients with and without renal response, respectively: baseline median eGFR (13.3 versus 9.3 mL/min/1.73 m(2), P = 0.017), Acute Kidney Injury Network Stage 3 (68.8 versus 92.1%, P = 0.019), haematological response rate (94 versus 34%, P < 0.0001), median percentage of free light chain (FLC) reduction at Day 21 (92 versus 24%, P = 0.006) and median number of casts/10 fields (14 versus 25, P = 0.005). The extent of interstitial fibrosis and tubular atrophy was similar. In multivariate analysis, only FLC reduction at Day 21 was significantly associated with renal response. However, when considering only the subgroup of haematological responders, both median number of casts [odds ratio (OR) = 0.93, 95% confidence interval (95% CI): 0.88-0.98, P = 0.01] and extent of tubular atrophy (OR = 0.03, 95% CI: 0.00-0.52, P = 0.02) were independent predictors of renal response.
In MCN, the presence of numerous casts and diffuse tubular atrophy is associated with poor renal prognosis. These data suggest that additional strategies to reduce FLC burden should be considered in patients with extensive cast formation.
轻链型骨髓瘤相关的 casts 肾病(MCN)是多发性骨髓瘤患者发生肾衰竭的主要原因,严重影响患者的生存。肾脏活检在 MCN 管理中的作用尚不清楚。
回顾性研究了 70 例多发性骨髓瘤伴 MCN 患者的肾脏病理发现。根据估计肾小球滤过率(eGFR)≥30 mL/min/1.73 m²和/或 3 个月时无需透析定义的肾脏反应是否实现,将患者分为有反应和无反应两组。
32 例(46%)患者实现了肾脏反应。在整个研究人群中,有反应和无反应患者之间存在以下显著差异:基线中位 eGFR(13.3 与 9.3 mL/min/1.73 m²,P=0.017)、急性肾损伤网络分期 3(68.8 与 92.1%,P=0.019)、血液学反应率(94 与 34%,P<0.0001)、第 21 天游离轻链(FLC)降低的中位数百分比(92 与 24%,P=0.006)和中位每 10 个视野的 casts 数(14 与 25,P=0.005)。间质纤维化和肾小管萎缩的程度相似。多变量分析表明,只有第 21 天的 FLC 降低与肾脏反应显著相关。然而,当仅考虑血液学反应者亚组时,cast 数量的中位数[比值比(OR)=0.93,95%置信区间(95%CI):0.88-0.98,P=0.01]和肾小管萎缩的程度(OR=0.03,95%CI:0.00-0.52,P=0.02)均是肾脏反应的独立预测因素。
在 MCN 中,大量 casts 的存在和弥漫性肾小管萎缩与不良的肾脏预后相关。这些数据表明,在广泛形成 casts 的患者中,应考虑采用额外的策略来降低 FLC 负荷。