Gogovska L, Ljapcev R, Polenakovic M, Stojkovski L, Popovska M, Grcevska L
Clinic of Neurology, Clinical Centre, Skopje, Republic of Macedonia.
Int J Artif Organs. 2003 Feb;26(2):170-3. doi: 10.1177/039139880302600212.
All patients with thymomatous Myasthenia Gravis (MG) should undergo early and total thymectomy, but controversy abounds in the choice of chronic immunosuppressive agents. The value of plasmaexchange (PE) in MG has been clearly established in preoperative preparation and treatment of myasthenic crisis. Whether PE may be used in the chronic long-term therapy of patients with thymomatous MG in addition to conventional immunosuppressive agents and cholinesterase inhibitors is yet to be answered.
We present a 40-year old woman with an 11 year history of MG. Thymectomy was done during the first year of the disease and the histopathologic finding was thymoma. To sustain clinical remission after thymectomy she continued with immunosuppression with methylprednisolone and cyclosporin A (or azathioprine) in addition to cholinesterase inhibitors. Despite the almost continuous immunosuppression, the disease course continued with fluctuating myasthenic weakness which few times progressed to myasthenic crisis requiring mechanical ventilation. During myasthenic crisis we performed 6-8 plasmapheresis at 2-3 day intervals in addition to conventional immunosuppressive therapy. The disease rapidly worsened in January 2000 and we started with intermittent plasmapheresis (3-6 procedures at 2-3 day intervals, every 6-8 weeks) in order to sustain remission. With this therapeutic protocol, during 20 months follow-up we managed to prevent myasthenic crisis and to avoid ventilatory support.
Plasmaexchange could be used as a successful and safe therapeutic tool in chronic long-term therapy in addition to conventional immunosuppressive agents to sustain remission in patients with MG. This is particularly important in the treatment of patients with thymomatous MG because they have an increased frequency of myasthenic crisis and often respond poorly an to immunosuppression with steroids or other immunosuppressants.
所有胸腺瘤型重症肌无力（MG）患者均应尽早接受全胸腺切除术，但在慢性免疫抑制剂的选择上存在诸多争议。血浆置换（PE）在MG的术前准备和肌无力危象的治疗中的价值已得到明确证实。除传统免疫抑制剂和胆碱酯酶抑制剂外，PE是否可用于胸腺瘤型MG患者的慢性长期治疗仍有待解答。
我们报告一名40岁女性，患有MG 11年。在疾病的第一年进行了胸腺切除术，组织病理学检查发现为胸腺瘤。为维持胸腺切除术后的临床缓解，除胆碱酯酶抑制剂外，她继续使用甲泼尼龙和环孢素A（或硫唑嘌呤）进行免疫抑制治疗。尽管几乎持续进行免疫抑制，但疾病进程仍表现为肌无力症状波动，有几次进展为需要机械通气的肌无力危象。在肌无力危象期间，除常规免疫抑制治疗外，我们每隔2 - 3天进行6 - 8次血浆置换。2000年1月病情迅速恶化，我们开始进行间歇性血浆置换（每隔2 - 3天进行3 - 6次，每6 - 8周一次）以维持缓解。通过这种治疗方案，在20个月的随访中，我们成功预防了肌无力危象并避免了通气支持。
除传统免疫抑制剂外，血浆置换可作为一种成功且安全的治疗手段用于MG患者的慢性长期治疗以维持缓解。这在胸腺瘤型MG患者的治疗中尤为重要，因为他们发生肌无力危象的频率增加，且对类固醇或其他免疫抑制剂的免疫抑制反应往往较差。
