组织化学和免疫组织化学技术对脂质沉积性肌病和糖原沉积性肌病的鉴别

目的脂质沉积性肌病和糖原沉积性肌病是罕见的肌肉疾病,其常规病理形态十分相似。本文探讨这两种疾病组织化学和免疫组化反应,寻找其鉴别诊断的形态差异。方法采用三磷酸腺苷(ATP)酶、还原性尼克酰胺腺嘌呤二核苷酸(NADH-Tr)、苏丹Ⅲ、PAS组织化学染色和抗肌萎缩蛋白(Dystrophin)免疫组化染色,分析2例脂质沉积性肌病和2例糖原沉积性肌病的组织形态特征和差别。结果脂质沉积性肌病和糖原沉积性肌病在常规HE染色均表现为肌纤维内出现大量的空泡,但前者空泡大小较一致,且边界清楚,后者空泡大小差异大,边界不清。ATP酶组化染色显示脂质沉积性肌病出现空泡变性的肌纤维均为Ⅰ型肌纤维,而糖原沉积性肌病出现空泡变性的肌纤维两型均有,以Ⅰ型严重。脂肪染色和糖原染色可作为这两种肌病的确诊依据。Dystrophin免疫组化染色显示脂质沉积性肌病的肌纤维强阳性,而在糖原沉积性肌病的反应为不连续弱阳性。结论组化和免疫组化检测可用于脂质沉积性肌病和糖原沉积性肌病的鉴别诊断。

Pathologic differential diagnosis of lipid storage myopathy from glycogen storage myopathy by histochemistry and immunohistochemitry

Objective To explore the reaction of histochemical and immunohistochemical staining in both myopathies, and investigate their morphologic difference. Methods By using ATPase, NADH-Tr, Sudan III and PAS histochemitry and dystrophin immuneohistochemitry, we analyzed the morphologic characterization and difference of 2 cases of lipid storage myopathies and 2 cases of glycogen storage myopathies. Results Muscular fibers contain numerous vacuoles in both lipid storage and glycogen storage myopathy. However, the vacuoles appeared similar in size and had a clear margin in the former diseases, while the vacuole varies in size and had an unclear margin in the later. ATPase staining showed that the vacuoles were merely in type I fibers in lipid storage myopathy, but both type muscular fibers contained vacuoles in glycogen storage myopathy with more predominant involve in type I fibers. The lipid and glycogen stainings could be used as markers for differential diagnosis. The muscular fibers had a strong immunoreaction in lipid storage myopaphy, while an illegible reaction was seen in the fibers in glycogen storage myopathy. Conclusion It is meaningful using histochemical and immuneohistochemical staining in differentiating lipid storage from glycogen storage myopathy.