Bornemann A, Kirchner T
Institute of Pathology, University of Erlangen-Nürnberg, Germany.
Virchows Arch. 1998 Apr;432(4):357-61. doi: 10.1007/s004280050178.
Thymic myoid cells (TMC) are sparse muscle-like cells in the thymic medulla, which are believed to trigger the autoimmune response in myasthenia gravis (MG). Ultrastructural investigations have revealed mature, degenerating, and immature TMC, but the number of TMC in MG patients does not differ from that in controls. We examined the turnover of TMC at the subcellular level, performing an immunocytochemical study with muscle-specific anti-desmin labelling of 10 thymuses derived from MG patients with lymphofollicular hyperplasia and from 8 normal controls. All thymuses examined revealed mature, immature, and degenerating TMC. Mature TMC contained desmin filaments in between Z-discs provided the sarcomeres were arranged in register. Morphological features of degenerating TMC included hypercontracted sarcomeres, cytoplasmic granular debris, chromatin clumping and, occasionally, membrane-bound bodies. Macrophages were not involved in the process. Immature TMC were of small diameter and contained myofilaments not arranged in myofibrils. In an MG thymus, small immature TMC were found clustered with dying TMC. It may be that degeneration of TMC is a stimulus for the generation of new TMC with faster turnover. This mechanism may mean that more antigen is available in MG patients than in normal controls, despite constant TMC numbers.
胸腺肌样细胞(TMC)是胸腺髓质中稀疏的肌样细胞,被认为可引发重症肌无力(MG)的自身免疫反应。超微结构研究揭示了成熟、退化和未成熟的TMC,但MG患者的TMC数量与对照组并无差异。我们在亚细胞水平上研究了TMC的更新情况,对10例患有淋巴滤泡增生的MG患者和8例正常对照的胸腺进行了肌肉特异性抗结蛋白标记的免疫细胞化学研究。所有检查的胸腺均显示出成熟、未成熟和退化的TMC。如果肌节排列整齐,成熟的TMC在Z盘之间含有结蛋白丝。退化的TMC的形态学特征包括肌节过度收缩、细胞质颗粒碎片、染色质聚集,偶尔还有膜结合体。巨噬细胞未参与此过程。未成熟的TMC直径较小,含有未排列成肌原纤维的肌丝。在MG胸腺中,发现小的未成熟TMC与濒死的TMC聚集在一起。可能TMC的退化是产生更新更快的新TMC的刺激因素。尽管TMC数量恒定,但这种机制可能意味着MG患者比正常对照有更多的抗原。