Lin Zi-Shan, Zhang Xu, Li Dan-Yang, Yu Xiao-Juan, Qin Ai-Bo, Dong Yujun, Zhou Fu-De, Wang Suxia, Zhao Ming-Hui
Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China.
Laboratory of Electron Microscopy, Pathological Center, Peking University First Hospital, Beijing, China.
J Clin Pathol. 2021 Jun 29. doi: 10.1136/jclinpath-2021-207449.
A varying proportion of patients with multiple myeloma suffer from more than one type of kidney disease simultaneously, of which the most common pattern is coexistent light chain cast nephropathy and light chain deposition disease (LCCN+LCDD). We investigated clinicopathological characteristics and outcomes of LCCN+LCDD in comparison with pure LCCN and pure LCDD.
We retrospectively analysed 45 newly diagnosed multiple myeloma patients with pure LCCN (n=26), LCCN +LCDD (n=9) and pure LCDD (n=10) between 2000 and 2019 at Peking University First Hospital.
Pathologically, patients with LCCN+LCDD were more likely to have λ light chain isotype and presented atypical features of LCDD including less nodular glomerulosclerosis and less deposit distribution than patients with pure LCDD. In clinical characteristics, patients with LCCN +LCDD and patients with pure LCCN shared similar features. The death-censored renal survival in patients with LCCN +LCDD was similar to patients with pure LCCN but worse than patients with pure LCDD, but the overall survival was much better than patients with LCCN alone and similar to patients with pure LCDD. For patients with pure LCCN, the independent predictor of death-censored renal survival was lactate dehydrogenase, and the independent predictors of overall survival were the mean number of casts and serum albumin.
Patients with LCCN+LCDD had similar renal outcome compared with patients with pure LCCN but the overall survival is much better. Thus, for patients with LCCN, especially those with λ restriction, pathologists should carefully evaluate the kidney specimens to exclude the possibility of combined LCDD.
不同比例的多发性骨髓瘤患者同时患有不止一种类型的肾脏疾病,其中最常见的模式是轻链管型肾病和轻链沉积病并存(LCCN+LCDD)。我们比较了LCCN+LCDD与单纯LCCN和单纯LCDD的临床病理特征及预后。
我们回顾性分析了2000年至2019年期间北京大学第一医院45例新诊断的多发性骨髓瘤患者,其中单纯LCCN患者26例,LCCN+LCDD患者9例,单纯LCDD患者10例。
病理上,与单纯LCDD患者相比,LCCN+LCDD患者更易出现λ轻链亚型,且呈现LCDD的非典型特征,包括结节性肾小球硬化较少和沉积物分布较少。在临床特征方面,LCCN+LCDD患者与单纯LCCN患者有相似特征。LCCN+LCDD患者的死亡校正肾脏生存率与单纯LCCN患者相似,但比单纯LCDD患者差,但其总生存率比单纯LCCN患者好得多,与单纯LCDD患者相似。对于单纯LCCN患者,死亡校正肾脏生存率的独立预测因素是乳酸脱氢酶,总生存率的独立预测因素是管型平均数和血清白蛋白。
LCCN+LCDD患者与单纯LCCN患者的肾脏预后相似,但总生存率要好得多。因此,对于LCCN患者,尤其是那些有λ限制的患者,病理学家应仔细评估肾脏标本,以排除合并LCDD的可能性。