喉淀粉样变性病的临床报告并文献复习

目的：进一步认识喉淀粉样变性病的发病机制、分类、临床表现、诊断及治疗。方法：11例喉淀粉样变性病患者，行间接喉镜下手术5例，支撑喉镜下手术1例，喉裂开术2例，垂直半喉切除声门重建术1例，未手术2例。结果：3例失访，2例复发，6例情况稳定。结论：①Symmers分类方法具有临床价值；②诊断、鉴别诊断依据刚果红染色呈棕红色，偏光显微镜下呈双折光和绿荧光的特殊形态；③喉淀粉样变性确诊后，必须排除全身性、继发性淀粉样变性及多发性浆细胞瘤；④治疗依赖患者的严重程度，轻者可以保守观察，重者必须外科切除；⑤可以复发，必须长期随访。

Cases report and literature review of laryngeal amyloidosis

OBJECTIVE: To have a better understand about mechanism, classification, clinical manifestation, diagnosis and treatment of the laryngeal amyloidosis. METHOD: Among 11 cases of clinical material of patients suffering from laryngeal amyloidosis, 5 cases operated by indirect laryngoscope, 1 case by direct laryngoscope, 2 cases by laryngofissure, 1 case by vertical hemilaryngectomy with glottis reconstruction, 2 cases weren't operated. RESULT: Three cases lost follow-up, 2 cases were recurred, and 6 cases' condition remained stable. CONCLUSION: (1) Symmers' classification is generally accepted and has clinical value; (2) The Congo red staining is the classic and most specific histochemical test, the fibrils demonstrate characteristic apple-green birefringence when examined under polarized light microscopy, which provide basis on confirming the diagnosis. (3) Laryngeal amyloidosis usually indicated localized disease with systemic and secondary involvement uncommon, however, potential for an underlying disease state such as multiple myeloma, a full medical evaluation for systemic disease should be performed. (4) Management depends on the severity of the symptoms in regard to the individual patients and in some case, can be conservative observation. If disease is causing significant symptom or showing signs of increasing in size, surgical intervention must be carried out. (5) Recurrence maybe become manifested after several years and long-term follow-up is recommended.