Zhang C L, Feng J, Shen K N, Su W, Zhang C L, Huang X F, Cao X X, Zhang L, Zhou D B, Li J
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Nov 14;37(11):942-945. doi: 10.3760/cma.j.issn.0253-2727.2016.11.003.
To investigate the diagnostic and prognostic values of serum free light chain (sFLC) in patients with primary light chain amyloidosis (pAL). Patients diagnosed with pAL between January 2009 and June 2015 at Peking Union Medical College Hospital were included in this study to retrospectively evaluate the clinical data, sFLC, treatment and survival. In total, 126 newly diagnosed pAL patients with complete sFLC data were included in this study. The median age was 57 years old (range, 37-81 years) and male to female ratio was 1.57. Eighty patients (63.5%) had lambda type light chain. Renal and cardiac involvements occurred in 87 cases (69% ) and 79 cases (62.7% ), respectively. The median value of difference between involved and uninvolved serum immunoglobulin free light chain levels (dFLC) was 99 mg/L (range, 1-4 263 mg/L). The positive rate of serum protein electrophoresis, serum immune fixation electrophoresis, urine immunofixation electrophoresis and abnormal sFLC ratio to detect monoclonal immunoglobulin were 34.9%(44 cases), 63.5%(80 cases), 77.0%(97 cases) and 81.0% (102 cases), respectively. The sensitivity for identifying monoclonal immunoglobulin could be increased to 98.4%(124 cases) by using the above-mentioned four methods. The median follow-up was 16 months, 37 patients were dead at the last follow-up. The median overall survival of all patients were not reached. Multivariate analysis showed that dFLC≥130 mg/L was independently associated with inferior survival (=3.272, 95% 1.384-7.739, =0.007). Serum FLC measurement combined with other methods could improve the sensitivity of detection of monoclonal immunoglobulin in patients with pAL. The high level of dFLC was an independent prognostic factor for survival.
探讨血清游离轻链（sFLC）在原发性轻链型淀粉样变性（pAL）患者中的诊断及预后价值。本研究纳入2009年1月至2015年6月在北京协和医院诊断为pAL的患者，对其临床资料、sFLC、治疗及生存情况进行回顾性评估。本研究共纳入126例新诊断的pAL患者，其sFLC数据完整。中位年龄为57岁（范围37 - 81岁），男女比例为1.57。80例（63.5%）患者为λ型轻链。肾脏和心脏受累分别发生于87例（69%）和79例（62.7%）患者。受累与未受累血清免疫球蛋白游离轻链水平差值（dFLC）的中位值为99 mg/L（范围1 - 4263 mg/L）。血清蛋白电泳、血清免疫固定电泳、尿免疫固定电泳及异常sFLC比例检测单克隆免疫球蛋白的阳性率分别为34.9%（44例）、63.5%（80例）、77.0%（97例）和81.0%（102例）。采用上述四种方法可使识别单克隆免疫球蛋白的敏感性提高至98.4%（124例）。中位随访时间为16个月，末次随访时37例患者死亡。所有患者的中位总生存期未达到。多因素分析显示，dFLC≥130 mg/L与较差的生存独立相关（=3.272，95% 1.384 - 7.739，=0.007）。血清FLC检测联合其他方法可提高pAL患者单克隆免疫球蛋白的检测敏感性。dFLC高水平是生存的独立预后因素。
