王林, 岳书玲, 黄玲, 杨光, 李永强. 淀粉样管型肾病的临床病理特征分析[J]. 临床肾脏病杂志, 2023, 23(4): 292-299. DOI: 10.3969/j.issn.1671-2390.2023.04.003
    引用本文: 王林, 岳书玲, 黄玲, 杨光, 李永强. 淀粉样管型肾病的临床病理特征分析[J]. 临床肾脏病杂志, 2023, 23(4): 292-299. DOI: 10.3969/j.issn.1671-2390.2023.04.003
    shu
    Wang Lin, Yue Shu-ling, Huang Ling, Yang Guang, Li Yong-qiang. Clinicopathological characteristics of light chain amyloid cast nephropathy[J]. Journal of Clinical Nephrology, 2023, 23(4): 292-299. DOI: 10.3969/j.issn.1671-2390.2023.04.003
    Citation: Wang Lin, Yue Shu-ling, Huang Ling, Yang Guang, Li Yong-qiang. Clinicopathological characteristics of light chain amyloid cast nephropathy[J]. Journal of Clinical Nephrology, 2023, 23(4): 292-299. DOI: 10.3969/j.issn.1671-2390.2023.04.003
    shu

    淀粉样管型肾病的临床病理特征分析

    Clinicopathological characteristics of light chain amyloid cast nephropathy

      摘要
    • 摘要:
      目的  探讨轻链淀粉样管型肾病（light chain amyloid casts，LCAC）的临床病理学特征，加深对LCAC的认识。
      方法  回顾2018年1月至2021年6月在广州金域医学检验中心肾脏病理诊断为LCAC的患者，将患者分为单纯LCAC（pure light chain amyloid casts，I-LCAC）组和LCAC合并轻链淀粉样变性肾病（light chain amyloid casts with light chain amyloid nephropathy，LCAC&AL）组，所有患者均进行了光镜、免疫荧光、电镜和免疫电镜检查，对两组患者的肾脏病理进行半定量评分进行分析，评估两组患者的临床病理学特征。
      结果  同期诊断为轻链管型肾病（light chain cast nephropathy，LCCN）的患者共131例，其中15例诊断为LCAC（11.5%），包括I-LCAC组10例和LCAC&AL组5例；LCAC有9例临床确诊为多发性骨髓瘤，其中I-LCAC组7例，LCAC&AL组2例，两组差异无统计学意义；与LCAC&AL组相比，I-LCAC组患者血肌酐水平（766.1 ± 472.5）μmol/L比（82.8 ± 22.5）μmol/L和发生贫血的比例（100%比40%）更高（P<0.05）；I-LCAC组患者的肾组织内每平方毫米的平均淀粉管型数（7.3 ± 5.0）个/mm²比（0.3 ± 0.1）个/mm²显著多于LCAC&AL组（P<0.05），管型的轻链类型以λ型多见；每平方毫米的平均淀粉管型数与患者的血肌酐水平呈正相关（R2=0.593）。
      结论  LCAC的病因以多发性骨髓瘤最常见，管型多呈同心圆和分层状，中心区域染色较浅，而边缘嗜银呈“睫毛状”的病理形态特征和刚果红染色阳性，偏振光镜下有黄绿色双折光的染色特性，I-LCAC病理类型患者较LCAC&AL组更容易肾功能损伤，每平方毫米的平均淀粉管型数越多肾功能越差。

       

      Abstract:
      Objective  To explore the clinicopathological features of light chain amyloid casts (LCAC) and grasp a deeper understanding of LCAC.
      Methods  After reviewing the profiles of renal pathology at Guangzhou Kingmed Medical Laboratory Center from January 2018 to June 2021, LCAC patients were assigned into two groups of pure light chain amyloid casts (I-LCAC) and LCAC plus light chain amyloid nephropathy (LCAC&AL) group. All specimens were examined by light microscopy, immunofluorescence, electron microscopy and immunoelectron microscopy through semi-quantitative scoring.
      Results  A total of 131 patients were diagnosed with light chain cast nephropathy (LCCN) during the same period. Among which, 15 cases belonged to LCAC (11.5%), including I-LCAC group (n=10) and LCAC&AL group (n=5); 9 cases of LCAC were identified as multiple myeloma, including I-LCAC group (n=7) and LCAC&AL group (n=2) and the inter-group difference was not statistically significant; As compared with LCAC&AL group, I-LCAC group had higher serum creatinine levels (766.1±472.5)μmol/Lvs (82.8±22.5)μmol/L and incidence of anemia (100% vs 40%)(P<0.05); The mean number of starch casts per square millimeter in renal tissue of I-LCAC group was significantly more than that of LCAC&AL group (7.3±5.0)/mm²vs (0.3±0.1)/mm², P<0.05. The most common type of light chain was λ type. And the mean number of starch casts per square millimeter was correlated positively with serum creatinine level (R2=0.593).
      Conclusion  Multiple myeloma is the most common cause of LCAC. The casts are predominantly concentric and layered, central area is faintly stained while the edge of argyrophilic pathology appears "eyelash-like" with a positive Congo red stain. Polarized light microscope reveals yellow-green birefringence staining characteristics. Patients with I-LCAC pathological type are more susceptible to renal function injury than LCAC&AL group. The greater the mean number of starch tubes per square millimeter, the worse the renal function.

       

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