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Title: [The value of serum free light chain in differential diagnosis of monoclonal gammopathy of renal significance].
Author: Li C, Wen YB, Li H, Su W, Li J, Cai JF, Chen LM, Li XM, Li XW.
Journal: Zhonghua Yi Xue Za Zhi; 2017 Aug 08; 97(30):2344-2348. PubMed ID: 28822451.
Abstract:
Objective: To investigate the value of serum free light chain (FLC) in differential diagnosis of monoclonal gammopathy of renal significance (MGRS). Methods: Forty-nine hospitalized patients who underwent renal biopsy in Peking Union Medical College Hospital between January 2013 and December 2015 were included. Monoclonal gammopathy was detected by serum protein electrophoresis (SPE), serum immunofixation electrophoresis (IFE), urine IFE and serum FLC. All patients were classified as MGRS (n=32) and monoclonal gammopathy of undetermined significance (MGUS) (n=17). Results: Renal lesions in MGRS subgroup included light chain amyloidosis (n=24, 75.0%), light chain deposition disease (n=7, 21.9%), and fibrillary glomerulopathy (n=1, 3.1%). Renal diseases in MGUS subgroup included membranous nephropathy (n=10), focal segmental glomerulosclerosi (FSGS) (n=3), diabetic glomerulopathy (n=1), Henoch-Schonlein purpura nephritis (n=1), anti-GBM disease concurrent with membranous nephropathy (n=1) and glomerulomegaly (n=1). Positive number of SPE, serum IFE, urine IFE and abnormal number of serum FLC ratio in MGRS subgroup were 12, 16, 23 and 30, respectively. Positive number of SPE, serum IFE, urine IFE and abnormal number of serum FLC ratio in MGUS subgroup were 11, 17, 6 and 3, respectively. MGRS and MGUS subgroups differed significantly in positive rate of serum IFE (P<0.001), as well as positive rate of urine IFE (P=0.02) and abnormal rate of serum FLC ratio (P<0.001). The sensitivity, specificity, total consistent rate of serum FLC ratio for diagnosis of MGRS were 93.8%, 82.4%, and 89.8% respectively. The sensitivity for diagnosing MGRS could be increased to 100% by combining serum FLC ratio and urine IFE. Conclusions: The significance of monoclonal gammopathy in patients with renal disease should be evaluated by renal pathology.On the premise of excluding lymphoplasmacytic malignancy, serum FLC ratio had promising diagnostic value for MGRS, which was helpful for differential diagnosis of patients who had contraindication to renal biopsy. 目的： 明确血游离轻链(FLC)检测是否有助于鉴别诊断有肾脏意义的单克隆免疫球蛋白血症(MGRS)。 方法： 回顾性分析2013年1月至2015年12月在北京协和医院住院的49例单克隆免疫球蛋白血症患者资料，按照临床诊断和肾脏病理检查结果分为2组：MGRS组(32例)和意义未明的单克隆免疫球蛋白血症(MGUS)组(17例)。收集患者的血清蛋白电泳(SPE)、血免疫固定电泳(IFE)、尿IFE及血FLC检测结果。 结果： MGRS组肾脏病理类型：原发性轻链型淀粉样变性(pAL)24例(75.0%)，轻链沉积病(LCDD)7例(21.9%)，纤维样肾小球病1例(3.1%)。MGUS组肾脏病理类型：膜性肾病10例，局灶节段性肾小球硬化3例，糖尿病肾小球病、过敏性紫癜性肾炎、抗肾小球基底膜病合并膜性肾病、肾小球肥大各1例。MGRS组SPE、血IFE、尿IFE阳性及血FLC比值异常者分别为12例(12/29)、16例、23例、30例。MGUS组的SPE、血IFE，尿IFE阳性率及血FLC比值异常者分别为11例、17例、6例和3例。两组的血IFE阳性率、尿IFE阳性率及血FLC比值异常率的差异均有统计学意义(P<0.001，P＝0.02，P<0.001)。血FLC比值异常诊断MGRS的敏感度93.8%，特异度为82.4%，总符合率89.8%。血FLC比值异常联合尿IFE诊断MGRS的敏感度100%，特异度64.7%，总符合率87.8%。 结论： 肾脏病患者出现单克隆免疫球蛋白血症，需要结合肾活检病理确定其意义。在排除淋巴浆细胞肿瘤的前提下，血FLC比值异常对于MGRS有较高的诊断价值，有助于存在肾活检禁忌证的单克隆免疫球蛋白血症合并肾脏病患者病因的鉴别诊断。.

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