Vani Bharani, Ritu Aggarwal, Praveen Sharma, Pankaj Malhotra, Minz Ranjana
1Department of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
2Department of Immunopathology, Postgraduate Institute of Medical Education and Research, 4th Floor, Research Block A, Chandigarh, India.
Indian J Hematol Blood Transfus. 2018 Oct;34(4):632-635. doi: 10.1007/s12288-018-0926-0. Epub 2018 Jan 31.
Serum and urine protein electrophoresis and immunofixation are essential for identification and categorization of M protein/monoclonal protein. Based on the number of discrete bands identified the condition can be a monoclonal, biclonal or triclonal gammopathy. A subset of cases show an interesting pattern on immunofixation electrophoresis, with a complete immunoglobulin molecule, along with excess free light chains where in one light chain band shows similar mobility as the heavy chain, while the other light chain band of same isotype has a different mobility. Over a 3 year study period, 420 immunofixation electrophoresis gels were studied to select the cases with the typical pattern as described. The clinical records where searched for data of baseline evaluations done prior to starting therapy, including clinical presentation, biochemical parameters, hemogram and bone marrow examination. Twenty cases (4.7%) were identified from the records, of these 77.8% cases had renal impairment and 33.3% presented with rapidly progressive renal failure. The possible explanation is the toxic effects of excess free light chains, in our cohort. The bound LCs show mobility similar to the HC bands on serum immunofixation gels, however the free light chains, exist in polymeric forms and show a different mobility. The identification and reporting of this pattern provides additional information regarding the high load of light chains, and indicates that patient may have a poor renal outcome/performance.
血清和尿蛋白电泳及免疫固定电泳对于M蛋白/单克隆蛋白的鉴定和分类至关重要。根据所鉴定的离散条带数量,该病症可为单克隆、双克隆或三克隆性丙种球蛋白病。一部分病例在免疫固定电泳上呈现出有趣的模式,即存在完整的免疫球蛋白分子以及过量的游离轻链,其中一条轻链带的迁移率与重链相似,而同一同种型的另一条轻链带具有不同的迁移率。在为期3年的研究期间,对420份免疫固定电泳凝胶进行了研究,以挑选出具有上述典型模式的病例。查阅临床记录以获取开始治疗前进行的基线评估数据,包括临床表现、生化参数、血常规和骨髓检查。从记录中鉴定出20例(4.7%),其中77.8%的病例有肾功能损害,33.3%表现为快速进展性肾衰竭。在我们的队列中,可能的解释是过量游离轻链的毒性作用。结合的轻链在血清免疫固定凝胶上的迁移率与重链条带相似,然而游离轻链以聚合形式存在并表现出不同的迁移率。这种模式的鉴定和报告提供了有关轻链高负荷的额外信息,并表明患者可能有不良的肾脏预后/表现。