Steiner Normann, Schwärzler Angelika, Göbel Georg, Löscher Wolfgang, Wanschitz Julia, Gunsilius Eberhard
Department of Internal Medicine V (Hematology and Medical Oncology), Medical University of Innsbruck, A-6020 Innsbruck, Austria.
Department of Medical Statistics, Informatics and Health Economics, Medical University of Innsbruck, A-6020 Innsbruck, Austria.
Oncotarget. 2017 Jan 17;8(3):5081-5091. doi: 10.18632/oncotarget.13861.
Monoclonal gammopathy of undetermined significance (MGUS) is a premalignancy preceding multiple myeloma (MM) or related disorders. Neurological symptoms caused by the monoclonal immunoglobulins or free light-chains are often associated with a high morbidity. We analyzed the prevalence of neuropathy, clinical features and the long-term outcome in 223 patients (pts.) with MGUS.
Between 1/2005 and 3/2015, 223 adult pts. with MGUS were identified in our database.
In36/223 pts. (16%) a neuropathy was diagnosed (MGUS associated neuropathy, MGUS-N). 20 pts. (55%) had a distal symmetric axonal neuropathy, 10 pts. (28%) had a chronic inflammatory demyelinating polyneuropathy and 6 pts (17%) a distal acquired demyelinating symmetric polyneuropathy. In MGUS-NN (without neuropathy) and in MGUS-N, progression to smoldering MM, MM or Waldenstrom's macroglobulinemia (WM) occurred in 17% of the pts. The Immunoglobulin subtype was predominantly IgG in MGUS-NN and IgM in MGUS-N and ≥5.5% plasma cells in the bone-marrow predicted progression to MM and AL-amyloidosis in MGUS-NN and to WM in MGUS-N (p<0.05).
Due to the substantial prevalence of neuropathies, MGUS pts. should be monitored carefully and referred to a specialized center if neurological symptoms occur.
意义未明的单克隆丙种球蛋白病（MGUS）是多发性骨髓瘤（MM）或相关疾病之前的一种癌前病变。由单克隆免疫球蛋白或游离轻链引起的神经症状通常发病率较高。我们分析了223例MGUS患者的神经病变患病率、临床特征及长期预后。
2005年1月至2015年3月期间，在我们的数据库中识别出223例成年MGUS患者。
223例患者中有36例（16%）被诊断为神经病变（MGUS相关性神经病变，MGUS-N）。20例（55%）患有远端对称性轴索性神经病变，10例（28%）患有慢性炎症性脱髓鞘性多发性神经病变，6例（17%）患有远端获得性脱髓鞘性对称性多发性神经病变。在MGUS-NN（无神经病变）和MGUS-N患者中，17%的患者进展为冒烟型MM、MM或华氏巨球蛋白血症（WM）。免疫球蛋白亚型在MGUS-NN中主要为IgG，在MGUS-N中主要为IgM，骨髓中≥5.5%的浆细胞预示MGUS-NN患者进展为MM和AL淀粉样变性，MGUS-N患者进展为WM（p<0.05）。
由于神经病变的患病率较高，MGUS患者应仔细监测，若出现神经症状应转诊至专科中心。
