Rudy Shannon F, Jeffery Caroline C, Damrose Edward J
Department of Otolaryngology-Head and Neck Surgery, Stanford School of Medicine, Stanford University, Stanford, California, U.S.A.
Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, University of Alberta, Edmonton, Alberta, Canada.
Laryngoscope. 2018 Mar;128(3):670-674. doi: 10.1002/lary.26846. Epub 2017 Sep 4.
OBJECTIVES/HYPOTHESIS: Amyloidosis represents a heterogeneous group of disorders marked by abnormal protein formation and deposition. Laryngeal amyloidosis is rare and classically thought to remain isolated with little risk of systemic involvement or associated malignancy. This study sought to further characterize differences in clinical characteristics between patients with laryngeal and nonlaryngeal amyloidosis.
Retrospective case-control study.
The Stanford Translational Research Integrated Database Environment was searched to identify patients with biopsy-confirmed laryngeal amyloidosis and patients with amyloidosis without laryngeal involvement on endoscopy. Mann-Whitney U and χ tests were used for statistical analysis.
Of 865 patients treated for amyloidosis between 1996 and 2016, 22 (2.5%) patients with biopsy-proven laryngeal amyloidosis were identified. An additional 22 patients with amyloidosis of a different organ and negative laryngoscopy-and therefore without laryngeal amyloidosis-were identified as controls. Compared to these controls with nonlaryngeal amyloidosis, patients with laryngeal amyloidosis were younger (mean age 52.8 years vs. 68.4 years, P < .0006), and 18% had additional organ involvement. Immunoglobulin light-chain amyloidosis was the most common subtype in both groups of patients. Eighty-six percent of patients with laryngeal amyloidosis required surgical excision, and of these patients, over 30% required multiple excisions.
There is a significant rate (18%) of multiorgan involvement in patients with laryngeal amyloidosis, which contradicts conventional concepts that this is an isolated disorder. This finding could have a significant impact on the evaluation and management of patients with laryngeal amyloidosis.
3b. Laryngoscope, 128:670-674, 2018.
目的/假设：淀粉样变性是一组异质性疾病，其特征为异常蛋白质的形成和沉积。喉淀粉样变性较为罕见，传统观点认为其通常局限于局部，很少有全身受累或伴发恶性肿瘤的风险。本研究旨在进一步明确喉淀粉样变性患者与非喉淀粉样变性患者临床特征的差异。
回顾性病例对照研究。
检索斯坦福转化研究综合数据库环境，以识别经活检证实的喉淀粉样变性患者以及内镜检查显示无喉受累的淀粉样变性患者。采用曼-惠特尼U检验和χ检验进行统计分析。
在1996年至2016年间接受淀粉样变性治疗的865例患者中，确定了22例（2.5%）经活检证实的喉淀粉样变性患者。另外确定了22例不同器官淀粉样变性且喉镜检查阴性（即无喉淀粉样变性）的患者作为对照。与这些非喉淀粉样变性对照患者相比，喉淀粉样变性患者更年轻（平均年龄52.8岁对68.4岁，P <.0006），且18%有其他器官受累。免疫球蛋白轻链淀粉样变性是两组患者中最常见的亚型。86%的喉淀粉样变性患者需要手术切除，其中超过30%的患者需要多次切除。
喉淀粉样变性患者存在多器官受累的显著比例（18%），这与该疾病为局限性疾病的传统观念相矛盾。这一发现可能对喉淀粉样变性患者的评估和管理产生重大影响。
3b。《喉镜》，2018年，第128卷，第670 - 674页。
