Department of Hematology, Skåne University Hospital, Malmö, Sweden.
Department of Clinical Chemistry, Skåne University Hospital, Malmö, Sweden.
Scand J Clin Lab Invest. 2021 Sep;81(5):385-388. doi: 10.1080/00365513.2021.1929443. Epub 2021 Jun 7.
Monoclonal gammopathies involving immunoglobulin E (IgE) is a very rare phenomenon, with less than 70 cases being previously described in the literature. The IgE monoclonal gammopathies include malignant plasma cell disorders such as IgE multiple myeloma (MM), as well as the associated premalignant condition IgE monoclonal gammopathy of undetermined significance (MGUS). We report a case of a 41-year-old woman presenting with an IgE kappa monoclonal protein following routine laboratory testing. Serum protein electrophoresis (SPEP) initially showed a monoclonal protein in the beta-2 fraction, at an estimated concentration of <4 g/L. Subsequent serum immunofixation electrophoresis (SIFE) including antisera to Ig heavy chains delta and epsilon confirmed the presence of an IgE kappa monoclonal protein. Analysis of serum free light chains (FLCs) showed increased levels of kappa FLC, resulting in an abnormally elevated kappa/lambda FLC ratio. No Bence-Jones proteinuria was present. Bone marrow aspiration showed 6% plasma cells, and no sign of myeloma-associated end-organ damage was evident. Consequently, the patient was diagnosed with IgE kappa MGUS. In the present report, the clinical characteristics of the patient are compared to previous descriptions of IgE monoclonal gammopathy. The report further emphasizes the importance of considering the presence of monoclonal IgD or IgE when SIFE shows a clear band positive for a light chain but is negative for Ig heavy chains gamma, alpha and mu.
涉及免疫球蛋白 E (IgE) 的单克隆丙种球蛋白病是一种非常罕见的现象,文献中以前仅描述了不到 70 例。IgE 单克隆丙种球蛋白病包括恶性浆细胞疾病,如 IgE 多发性骨髓瘤 (MM),以及相关的癌前状态 IgE 意义未明的单克隆丙种球蛋白病 (MGUS)。我们报告了一例 41 岁女性,在常规实验室检查后出现 IgE kappa 单克隆蛋白。血清蛋白电泳 (SPEP) 最初显示β-2 区有单克隆蛋白,估计浓度<4 g/L。随后进行的血清免疫固定电泳 (SIFE),包括针对 Ig 重链 delta 和 epsilon 的抗血清,证实存在 IgE kappa 单克隆蛋白。血清游离轻链 (FLC) 分析显示 kappa FLC 水平升高,导致异常升高的 kappa/lambda FLC 比值。无 Bence-Jones 蛋白尿。骨髓抽吸显示 6%的浆细胞,没有骨髓瘤相关的终末器官损伤的迹象。因此,该患者被诊断为 IgE kappa MGUS。在本报告中,将患者的临床特征与以前描述的 IgE 单克隆丙种球蛋白病进行了比较。该报告进一步强调了在 SIFE 显示明确的轻链带,但 Ig 重链 gamma、alpha 和 mu 为阴性时,考虑存在单克隆 IgD 或 IgE 的重要性。