Janzen R W, Lachenmayer L, Fischer K
Med Klin. 1977 Nov 11;72(45):1931-7.
6 years after resection and postirradiation of an "asymptomatic" thymoma, myasthenia gravis developed in a 46 year old man which improved within 6 months following initiation of immunosuppressive therapy with azathioprine. In a 28 year old man with incomplete operative removal of a metastasizing malignant thymoma, a myasthenia gravis appeared 8 weeks after surgery, i.e. before irradiation of the tumor relics. Myasthenia improved upon irradiation of the tumor relics and was no longer demonstrable one year after onset of its clinical signs. During the whole period of manifestation of the myasthenia, antibodies to skeletal muscle were deomonstrated in the first case, while, in the second case, in which antinuclear factors were present in the serum throughout the course of the myasthenia, muscular antibodies were detected only prefinally, i.e. in a stage without clinical symptoms of myasthenia. A review of the literature indicates that myasthenia gravis is a particularly frequent event in postthymectomy-syndromes (e.g. polymyositis, thyreoiditis, lupus erythematosus, hematological and dermatological syndromes). In postthymectomy-myasthenia, latency of manifestation, clinical distribution and its course may be rather variable. The problems of diagnosis, immunology and therapy of postthymectomy-myasthenia are discussed and general conclusions concerning postthymectomy-syndromes are drawn.
一名46岁男性在“无症状”胸腺瘤切除及放疗6年后出现重症肌无力,在开始使用硫唑嘌呤进行免疫抑制治疗后的6个月内症状有所改善。一名28岁男性,转移性恶性胸腺瘤手术切除不完全,术后8周即肿瘤残余放疗前出现重症肌无力。肿瘤残余放疗后肌无力症状改善,临床症状出现一年后肌无力不再明显。在第一例重症肌无力整个表现期可检测到骨骼肌抗体,而在第二例中,重症肌无力整个病程血清中均存在抗核因子,仅在终末期即无重症肌无力临床症状阶段检测到肌肉抗体。文献综述表明,重症肌无力是胸腺切除术后综合征(如多发性肌炎、甲状腺炎、红斑狼疮、血液学和皮肤病综合征)中特别常见的情况。胸腺切除术后重症肌无力的表现潜伏期、临床分布及其病程可能差异很大。本文讨论了胸腺切除术后重症肌无力的诊断、免疫学和治疗问题,并得出了关于胸腺切除术后综合征的一般结论。