【轻链淀粉样变性患者巨舌导致吞咽困难】。

Juan José Gómez-Piña, Dulce Mariana Quiroz, Amairani Sierra-Hernández, Sergio Alberto Mendoza-Álvarez, Olga Lidia Vera-Lastra
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引用次数: 0

摘要

简介:巨舌裂作为系统性淀粉样变性的临床表现是一种罕见的疾病,在所有类型的淀粉样变性中发生率不到9%。本报告的目的是提出一个诊断方法的患者与大舌,提供一个系统的方法,并考虑相关的诊断可能性在他们的评估。临床病例:我们提出的情况下,60岁的男子谁提出了一个逐步扩大的巨大的舌头六个月,导致吞咽困难和减少口腔开口。舌活检,组织病理学显示同质嗜酸性淀粉样物质。刚果红染色光镜下呈红色染色淀粉样物质,偏振光下呈苹果绿双折射。骨髓活检显示30%浆细胞,明确诊断为软组织淀粉样变。虽然它是一种良性病变,但局部淀粉样变应与全身性淀粉样变区分开来。结论:巨舌症患者的诊断途径复杂,既有内分泌原因,也有肿瘤原因,甚至有沉积原因;作为孤立体征的患者在其入路中是一个挑战,因为累及气道是这些患者要避免的主要并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Giant tongue leading to dysphagia in light chain amyloidosis patient].

Introduction: Macroglossia as a clinical manifestation of systemic amyloidosis is a rare condition, occurring in less than 9% of all types of amyloidosis. The aim of this report is to present the diagnostic approach of a patient with macroglossia, providing a systematic approach and considering relevant diagnostic possibilities during their evaluation.

Clinical case: We present the case of a 60-year-old man who presented with a progressively enlarging giant tongue for six months, causing dysphagia and reduced oral opening. A tongue biopsy was taken, which histopathologically exhibited homogenous eosinophilic amyloid-like material. Congo red staining showed amyloid material with red dye under light microscopy and apple-green birefringence under polarized light. Bone marrow biopsy showed 30% plasma cells, allowing for a definitive diagnosis of soft tissue amyloidosis. Although it is a benign lesion, localized amyloidosis should be differentiated from systemic forms.

Conclusions: The approach of patients with macroglossia is complex due to the diagnostic possibilities, from endocrinological causes, neoplastic, and even by deposit; Being an isolated sign in a patient is a challenge in its approach, because the involvement of the airway is the main complication to avoid in these patients.

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