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巨舌症,多发性骨髓瘤伴发系统性淀粉样变性的首发表现:病例报告。

Macroglossia, the first manifestation of systemic amyloidosis associated with multiple myeloma: Case report.

机构信息

Department of Oral Pathology and Medicine, School of Dentistry, University of Chile, Santiago, Chile; Therapeutic Diagnostic Center Odontology and Pathological Anatomy Service, Hospital Complex San José, Santiago, Chile.

Department of Oral Pathology and Medicine, School of Dentistry, University of Chile, Santiago, Chile.

出版信息

J Stomatol Oral Maxillofac Surg. 2018 Dec;119(6):514-517. doi: 10.1016/j.jormas.2018.06.009. Epub 2018 Jun 25.

Abstract

Amyloidosis is a group of diseases characterized by an irreversible and extracellular deposition of fibrillar, amorphous protein known as amyloid in different organs and tissues. Amyloid deposits may occur locally in tissues or may involve various organs, resulting in a wide range of clinical manifestations. Amyloidosis of the head and neck is rarely seen and can reflect some plasma cell dyscrasia that affects B lymphocytes. Deposition of amyloid on the tongue is very rare and accounts for less than 9% of all types of amyloidosis. Amyloid involvement of the tongue is almost always secondary to systemic amyloidosis. We report a 73-year-old female who presented with weight loss and macroglossia. Firstly, she was diagnosed only with amyloidosis of the tongue. Her general health condition was evaluated, revealing renal dysfunction, anemia, hypercalcemia, and hyperphosphatemia. The final diagnosis was systemic amyloidosis with multiple myeloma. The patient was referred for emergency hemodialysis and chemotherapy. Her condition progressed to congestive heart failure and recurrent urinary and respiratory infections. After 100 days from diagnosis, the patient died by pulmonary infection as a consequence of her weakened state of health. It is important to highlight role of the dentist especially oral pathologist to the evaluation of local alterations that may reflect systemic deterioration of patients.

摘要

淀粉样变性是一组疾病,其特征为在不同器官和组织中不可逆地且细胞外沉积纤维状、无定形的蛋白质,称为淀粉样物质。淀粉样物质沉积可局部发生在组织中,也可累及多个器官,导致广泛的临床表现。头颈部淀粉样变性罕见,可反映影响 B 淋巴细胞的一些浆细胞失调。舌部淀粉样物质沉积非常罕见,占所有淀粉样变性类型的不到 9%。舌部淀粉样物质沉积几乎总是继发于系统性淀粉样变性。我们报告了一位 73 岁女性,她因体重减轻和巨舌症就诊。首先,她仅被诊断为舌淀粉样变性。评估了她的一般健康状况,发现肾功能不全、贫血、高钙血症和高磷血症。最终诊断为多发性骨髓瘤伴系统性淀粉样变性。患者被转介进行紧急血液透析和化疗。她的病情进展为充血性心力衰竭和反复发生的尿路感染和呼吸道感染。从诊断到患者死亡,共 100 天,由于健康状况恶化,她死于肺部感染。重要的是要强调牙医特别是口腔病理学家在评估可能反映患者全身恶化的局部改变方面的作用。

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