原发性淀粉样变性的临床病理及电镜观察

The Clinicopathologjcal and Ultrastructural Study on Primary Amyloidosis

淀粉样变性是指淀粉样蛋白在皮肤、粘膜或内脏器官的细胞外沉积引起的一种少见疾病。口腔粘膜中舌粘膜是本病的好发部位。本组4例典型地发生于口腔颌面部的原发性淀粉样变性病例,3例病变部位为舌,1例为皮肤和口腔粘膜同时受累,临床提示有必要进行全身系统检查及追踪随访。并就其临床表现、常规病理、组织化学及电镜进行研究,同时讨论了淀粉样蛋白的光镜、电镜特点,组织学类型,生化组成及来源。

Amyloidosis is a rare disorder characterized by intercellular deposition of amyloid in various tissues of the body,such as skin,mucous membranes and internal organs. Of oral mucosa,periglottis is its predilection site. Four cases of typical oral and maxillofacial primary amyloidosis were reported, 3 cases had lesions on the tongue, 1 case had lesion involved in both skin and oral mucosa. It is essential to conduct general physical check-up and follow-up survey. The cases were also studied by clinical methodology, pathology, histochemistry and ultrastructural examination. The characteristics, types, biochemical composition and origin of amyloidosis were discussed in detail.