Mufarrij A A, Busaba N Y, Zaytoun G M, Gallo G R, Feiner H D
Department of Pathology, American University of Beirut Medical Center, Lebanon.
Am J Surg Pathol. 1990 Apr;14(4):379-83. doi: 10.1097/00000478-199004000-00011.
Primary localized amyloidosis of the nose and nasopharynx is a rare disease. We present a case and review seven additional cases from the English literature. The ages of the patients ranged from 8 to 86 years; there was no sex predominance. Symptoms were nasal obstruction, epistaxis, and impaired hearing. Physical examination revealed a nasal mass or glue ears. The lesions were composed of amyloid and chronic inflammatory cells, mainly plasma cells. Ours is the first case of nasal amyloidosis in which the type of amyloid was determined immunohistochemically to be amyloid light chain (AL) lambda. The main treatment was surgical. Recurrences developed. Determination of the biochemical nature of this amyloid clarified its pathogenesis and may influence treatment. Amyloidosis should be considered in the differential diagnosis of nasal obstruction, epistaxis, and glue ears, even in the pediatric age group.
鼻及鼻咽部原发性局限性淀粉样变性是一种罕见疾病。我们报告1例病例，并复习英文文献中的另外7例病例。患者年龄为8至86岁，无性别优势。症状为鼻塞、鼻出血及听力受损。体格检查发现鼻腔肿物或胶耳。病变由淀粉样物质和慢性炎症细胞（主要是浆细胞）组成。我们的病例是首例经免疫组织化学确定淀粉样物质类型为轻链（AL）λ型的鼻淀粉样变性。主要治疗方法为手术。出现了复发情况。对这种淀粉样物质生化性质的测定阐明了其发病机制，可能会影响治疗。即使在儿童年龄组，在对鼻塞、鼻出血及胶耳进行鉴别诊断时也应考虑淀粉样变性。
