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目的:探讨鼻、咽及喉部淀粉样变性的临床特点。方法:12例淀粉样变性病患者中,1例与多发性骨髓瘤有关的淀粉样变性者累及鼻咽部、喉部及面部,确诊后转血液科化疗;1例鼻部右下鼻甲浆细胞瘤伴淀粉样变性者在局部麻醉鼻内镜下切除后转血液科化疗;5例喉部声带息肉伴淀粉样变性者,行支撑喉镜下息肉及淀粉样变性组织切除术;3例局灶性双侧声带累及声门下气管病变者在支撑喉镜下分别用电刀接支撑喉镜息肉手术刀(外套塑料输液器绝缘)加(或)CO2激光切除术;2例咽部淀粉样变性者分别行全身麻醉下扁桃体切除术和直达喉镜用电刀接支撑喉镜息肉手术刀(外套塑料输液器绝缘)会厌舌面根部病变切除术。结果:1例与多发性骨髓瘤有关的淀粉样变性者18个月后死于恶病质伴肺炎及多器官功能衰竭;1例鼻部右下鼻甲浆细胞瘤伴淀粉样变性者随访2年无复发;5例喉部声带息肉伴淀粉样变性者随访1~3年无淀粉样变复发;3例局灶性双侧声带累及声门下气管病变者中,1例随访3年无复发,2例分别在术后4个月和6个月前连合及声门下局部复发1次,再行支撑喉镜下分别用电刀接支撑喉镜息肉手术刀切除术,随访6个月无复发;2例咽部淀粉样变性者(右侧扁桃体和会厌舌面根部)随访2年无复发。结论:鼻、咽及喉部淀粉样变性病因与多种因素有关,临床表现复杂缺乏特异性。在诊断、治疗过程中要注意区分原发性淀粉样变性(局部或全身性)、继发性淀粉样变性(局部或全身性)、与多发性骨髓瘤有关的淀粉样变性、遗传性或家族性淀粉样变性,减少误诊。
Objective: To investigate the clinical features of nasal, pharyngeal and laryngeal amyloidosis. Methods: Of the 12 patients with amyloidosis, 1 had amyloidosis associated with multiple myeloma involving the nasopharynx, larynx and face. After diagnosis, they were switched to hematology and chemotherapy. One case of nasal inferior right inferior turbinate cells Tumors with amyloidosis under local anesthesia endoscopic resection after hematology chemotherapy; 5 cases of vocal cord polyps with amyloidosis who underwent laryngoscope support polyp and amyloidosis resection; 3 cases of focal Sexual bilateral vocal cord involving the subglottic tracheal lesions were supported by laryngoscope with electric knife support laryngoscope polyp scalpel (jacket plastic infusion insulation) plus (or) CO2 laser resection; 2 cases of pharyngeal amyloidosis Under general anesthesia, respectively, tonsillectomy and direct laryngoscopy with electric knife support laryngoscope polyp scalpel (jacket plastic infusion set insulation) epiglottis tongue radical root excision. RESULTS: One case of amyloidosis associated with multiple myeloma died of cachexia with pneumonia and multiple organ failure after 18 months. One case of nasal inferior turbinate plasma cell tumor with amyloidosis was followed up for 2 years without recurrence ; 5 cases of vocal cord polyps with amyloidosis were followed up 1 to 3 years without amyloidosis recurrence; 3 cases of bilateral bilateral vocal fold involving the subglottic tracheal lesions, 1 case of follow-up 3 years without recurrence, 2 cases After 4 months and 6 months before the commissure and subglottic local recurrence 1 times, and then under the support laryngoscope were supported by electric knife laryngoscope polyp scalpel resection, no recurrence 6 months follow-up; 2 cases Pharyngeal amyloidosis (right tonsil and epiglottis tongue root) followed up for 2 years without recurrence. CONCLUSIONS: The causes of amyloidosis in the nose, pharynx and larynx are related to a variety of factors. The clinical manifestations are complex and lack specific. In the diagnosis, treatment should pay attention to distinguish between primary amyloidosis (local or systemic), secondary amyloidosis (local or systemic), associated with multiple myeloma amyloidosis, hereditary or family Amyloidosis, reduce misdiagnosis.