Pribitkin Edmund, Friedman Oren, O'Hara Brian, Cunnane Mary F, Levi David, Rosen Marc, Keane William M, Sataloff Robert T
Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University Hospital, 1721 Pine Street, Philadelphia, PA, USA.
Laryngoscope. 2003 Dec;113(12):2095-101. doi: 10.1097/00005537-200312000-00007.
OBJECTIVES/HYPOTHESIS: To delineate the clinical and pathologic characteristics of upper aerodigestive tract amyloidosis with particular attention to laryngeal amyloidosis.
Retrospective chart review of patients with amyloidosis of Thomas Jefferson University and its affiliated hospitals.
The charts of 16 patients with upper aerodigestive tract amyloidosis identified from the databases of the Thomas Jefferson University pathology department were reviewed and included in the study.
Sixteen patients (9 male and 7 female, with an average age of 49.8 years) with upper aerodigestive tract amyloidosis were identified. The most common site of amyloid involvement was the larynx. Consequently, patients most commonly presented with hoarseness (14 of 16). All patients underwent surgical removal of the amyloid deposits. Fourteen patients had primary localized amyloidosis. Two experienced systemic involvement. Seven of the 16 patients developed recurrences requiring further treatment.
Amyloidosis of the upper aerodigestive tract generally behaves as a benign, localized condition treatable by surgical resection. Regular follow-up with laryngoscopy is indicated for early diagnosis of recurrence, and multiple surgical procedures may be required to control symptoms.
目的/假设：明确上呼吸道消化道淀粉样变性的临床和病理特征，尤其关注喉淀粉样变性。
对托马斯·杰斐逊大学及其附属医院的淀粉样变性患者进行回顾性病历审查。
回顾并纳入了从托马斯·杰斐逊大学病理科数据库中确定的16例上呼吸道消化道淀粉样变性患者的病历。
确定了16例上呼吸道消化道淀粉样变性患者（9例男性，7例女性，平均年龄49.8岁）。淀粉样蛋白累及的最常见部位是喉部。因此，患者最常见的症状是声音嘶哑（16例中有14例）。所有患者均接受了淀粉样沉积物的手术切除。14例患者为原发性局限性淀粉样变性。2例出现全身受累。16例患者中有7例复发，需要进一步治疗。
上呼吸道消化道淀粉样变性通常表现为一种良性的局限性疾病，可通过手术切除治疗。建议定期进行喉镜检查随访，以便早期诊断复发，可能需要多次手术来控制症状。
