Laryngeal Amyloidosis

Laryngeal Amyloidosis
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喉部淀粉样变性

DOI:
10.1177/000348947708600624
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发表时间:
1977
期刊:
Annals of Otology, Rhinology & Laryngology
影响因子:
--
通讯作者:
Tasneem Zafar
Tasneem Zafar
中科院分区:
--
文献类型:
--
作者:
E. L. Barnes;Tasneem Zafar

文献摘要

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淀粉样变累及喉通常是一种孤立的现象,在全身性淀粉样变中很少发生。这项研究包括六名女性和一名男性,平均年龄为54岁,他们主要抱怨长期嘶哑。在直接喉镜下,淀粉样蛋白通常表现为坚硬的、无溃疡的黄色、红色或白色病变,最常累及脑室、真假声带。手术活检和/或切除是唯一的治疗方式。一名患者经历了她的淀粉样蛋白复发7年后,她的第一次手术,而另一名患者发展为喉外淀粉样蛋白。淀粉样蛋白以无定形嗜酸性物质的形式沉积在小唾液腺的血管壁和基底膜中,作为随机组织块,或作为脂肪组织中的"环"。在电子显微镜下,所有类型的淀粉样蛋白具有相同的基本结构。然而,化学分析揭示了三种不同类型的淀粉样蛋白:免疫球蛋白来源的淀粉样蛋白,未知来源的淀粉样蛋白和apudamyloid。有证据表明,至少在某些情况下,淀粉样蛋白定位于喉免疫球蛋白的起源。据推测,喉的单克隆浆细胞浸润的分泌物被邻近的组织细胞代谢成免疫球蛋白轻链片段,并作为淀粉样蛋白沉积在细胞外。
The larynx is usually involved with amyloid as an isolated phenomenon and infrequently in generalized amyloidosis. This study includes six females and one male with an average age of 54 years who complained chiefly of prolonged hoarseness. At direct laryngoscopy, the amyloid usually presented as firm, nonulcerated yellow, red, or white lesions most often involving the ventricle, true and false cords. Surgical biopsy and/or excision was the only form of treatment. One patient experienced recurrence of her amyloid seven years after her first operation while another patient developed extralaryngeal amyloid. Amyloid is deposited extracellularly as amorphous eosinophilic material in vascular walls and basement membranes of minor salivary glands, as random tissue masses, or as “rings” in adipose tissue. Under the electron microscope, all types of amyloid have the same basic structure. However, chemical analysis has revealed three different classes of amyloid: Amyloid of immunoglobulin origin, amyloid of unknown origin, and apudamyloid. Evidence suggests an immunoglobulin origin of at least some cases of amyloid localized to the larynx. It is theorized that the secretions of a monoclonal plasmacytic infiltrate of the larynx are metabolized into immunoglobulin light chain fragments by adjacent histiocytes and deposited extracellularly as amyloid.