Crystalline appearance in light chain cast nephropathy is associated with higher early mortality in patients with newly diagnosed multiple myeloma.
Crystalline appearance in light chain cast nephropathy is associated with higher early mortality in patients with newly diagnosed multiple myeloma.
复制标题
轻链管型肾病的结晶外观与新诊断的多发性骨髓瘤患者较高的早期死亡率相关。
DOI：
10.1016/j.intimp.2021.107875
复制
发表时间：
2021
影响因子：
5.6
通讯作者：
Ming
中科院分区：
文献类型：
--
作者：
Zi;Xu Zhang;Xiao;Shuang Wang;Su;Yuanli Dong;F. Zhou;Ming
轻链管型肾病（Light chain cast nephropathy，LCCN）是多发性骨髓瘤患者最常见的肾脏病变. LCCN可以表现出结晶外观。结晶LCCN的发生率和临床意义尚不清楚。在这里，我们报告的第一个回顾性研究结晶LCCN。MethodsTwenty 6例LCCN患者参加。我们研究了LCCN患者的临床病理特征和预后，并将普通LCCN患者（n= 18）与结晶LCCN患者（n= 8）进行了比较。LCCN患者的中位年龄为57.5岁（范围，41-75岁）。无患者出现肾病综合征。两组间临床特征无显著差异。所有晶体LCCN患者患有晚期多发性骨髓瘤和急性肾损伤。结晶型LCCN患者以λ型为主（7/8，87.5%）。普通型LCCN患者的肾小管萎缩和急性肾小管损伤评分显著高于结晶型LCCN患者。5例LCCN晶体管型抗人Tamm-Horsfall糖蛋白染色阴性。晶体LCCN组和普通LCCN组的中位总生存期无显著差异（6.0个月vs. 35.0个月，p= 0.173）。但晶体型LCCN患者的早期死亡率高于普通型LCCN患者（50.0%vs11.1%，p = 0.03）。因此，对于LCCN患者，应仔细筛查晶体外观。
BackgroundLight chain cast nephropathy (LCCN) is the most common kidney lesion in multiple myeloma patients. LCCN may exhibit a crystalline appearance. The frequency and clinical significance of crystalline LCCN are not well understood. Here, we report the first retrospective study of crystalline LCCN.MethodsTwenty-six patients with LCCN were enrolled. We studied the clinicopathological features and outcomes of LCCN patients and compared ordinary LCCN patients (n= 18) with crystalline LCCN patients (n= 8).ResultsCrystalline LCCN was not rare (8/26, 30.8%) in our study. The median age of LCCN patients was 57.5 (range, 41–75) years. No patients presented with nephrotic syndrome. No significant differences in clinical features were observed between the two groups. All crystalline LCCN patients suffered from advanced multiple myeloma and acute kidney injury. There was a dominance of the λ isotype (7/8, 87.5%) in patients with crystalline LCCN. Patients with ordinary LCCN had significantly higher scores of tubular atrophy and acute tubular injury than those with crystalline LCCN. The crystalline casts of 5 crystalline LCCN patients stained negative with antihuman Tamm-Horsfall glycoprotein. There were no significant differences in the median overall survival between the crystalline LCCN group and the ordinary LCCN group (6.0 months vs. 35.0 months,p= 0.173). However, crystalline LCCN patients had higher early mortality than ordinary LCCN patients (50.0% vs 11.1%,p= 0.03).ConclusionCrystalline LCCN patients had higher early mortality than ordinary LCCN patients. Thus, for patients with LCCN, crystalline appearance should be screened carefully.