Rech Jürgen, Hueber Axel J, Kallert Stefan, Leipe Jan, Kalden Jochen R, Beck Marcus, Schett Georg, Schulze-Koops Hendrik
Medical Department III, University of Erlangen-Nuremberg, Erlangen, Germany.
Ther Apher Dial. 2008 Jun;12(3):205-8. doi: 10.1111/j.1744-9987.2008.00573.x.
Demyelinating polyneuropathy with anti-myelin associated glycoprotein (anti-MAG) antibodies is an immune mediated disorder characterized by proximal and distal symmetric weakness. Electrophysiological measurements depict features characteristic for demyelination, including prolonged distal latency, retarded conduction velocity, delayed or absent F-waves, and, rarely, partial conduction block. We report on a 65-year-old patient who was diagnosed with demyelinating polyneuropathy and anti-MAG antibodies five years before admission. Despite immunosuppressive agents and extracorporeal therapy (plasmapheresis) the disease progressed as assessed by clinical symptoms and neurological tests. Laboratory results showed an increase of serum immunoglobulin M and anti-MAG antibodies over time. Because of progressive disease we decided to treat the patient with immunoadsorption followed by application of the anti-CD20 antibody, rituximab. Six cycles of selective immunoadsorption were performed over three-weekly intervals with a repetitively used column (Globaffin); each cycle consisted of four consecutive daily treatments. Starting at cycle 4 the anti-CD20 antibody rituximab was administered with 375 mg/m(2) after immunoadsorption. The pretreatment anti-MAG antibody level of 10,000 U/mL, indicating disease activity, initially increased during treatment to a maximum of 30,559 U/mL. However, after completion of the six cycles, the anti-MAG level had decreased to 2348 U/mL; 16 months after the last immunoadsorption cycle the anti-MAG level had increased to 4134 U/mL, while the conduction velocity and compound motor action potentials remained stabile. Immunoadsorption in combination with a monoclonal anti-CD20 antibody in patients with demyelinating polyneuropathy with anti-MAG is effective and can be used an alternative treatment option in patients with progressive disease.
伴有抗髓鞘相关糖蛋白（anti-MAG）抗体的脱髓鞘性多发性神经病是一种免疫介导的疾病，其特征为近端和远端对称性肌无力。电生理测量显示出脱髓鞘的特征，包括远端潜伏期延长、传导速度减慢、F波延迟或消失，以及罕见的部分传导阻滞。我们报告一例65岁患者，入院前五年被诊断为脱髓鞘性多发性神经病和anti-MAG抗体阳性。尽管使用了免疫抑制剂和体外治疗（血浆置换），但根据临床症状和神经学检查评估，疾病仍在进展。实验室结果显示血清免疫球蛋白M和anti-MAG抗体随时间增加。由于疾病进展，我们决定对患者进行免疫吸附治疗，随后应用抗CD20抗体利妥昔单抗。每隔三周使用重复使用的柱（Globaffin）进行六个周期的选择性免疫吸附；每个周期包括连续四天的治疗。从第4周期开始，在免疫吸附后给予抗CD20抗体利妥昔单抗，剂量为375 mg/m²。治疗前anti-MAG抗体水平为10,000 U/mL，表明疾病活动，治疗期间最初升高至最高30,559 U/mL。然而，六个周期完成后，anti-MAG水平降至2348 U/mL；最后一次免疫吸附周期后16个月，anti-MAG水平升至4134 U/mL，而传导速度和复合运动动作电位保持稳定。对于伴有anti-MAG的脱髓鞘性多发性神经病患者，免疫吸附联合单克隆抗CD20抗体是有效的，可作为疾病进展患者的替代治疗选择。
