We describe an unusual case of myasthenia gravis. Our patient had been diagnosed as having myasthenia gravis with thymoma at the age of 64 years, and died of acute respiratory failure at the age of 80 years. Post mortem examination revealed CD8-positive lymphocytic infiltration with numerous giant cells in the skeletal muscles and myocardium. Immunohistochemical and ultrastructural studies revealed that there were two types of giant cells: histiocytic and myocytic in origin. Furthermore, both types of giant cells were immunopositive for proteins implicated in the late endosome and lysosome-protease systems, suggesting that endocytosis may be the key mechanism in the formation of giant cells. The present case, together with a few similar cases reported previously, may represent a particular subset of polymyositis, that is, giant cell polymyositis and myocarditis associated with myasthenia gravis and thymoma.

译文

我们描述了一个不寻常的重症肌无力病例。我们的患者在64岁时被诊断为患有胸腺瘤的重症肌无力,并在80岁时死于急性呼吸衰竭。验尸后发现CD8-positive淋巴细胞浸润,骨骼肌和心肌中有许多巨细胞。免疫组织化学和超微结构研究表明,有两种类型的巨细胞: 组织细胞和肌细胞起源。此外,两种类型的巨细胞对与晚期内体和溶酶体-蛋白酶系统有关的蛋白质均具有免疫阳性,这表明内吞作用可能是巨细胞形成的关键机制。本病例以及先前报道的一些类似病例可能代表多发性肌炎的特定子集,即与重症肌无力和胸腺瘤相关的巨细胞多发性肌炎和心肌炎。

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