Arweiler D J, Jahnke K
Universitäts-HNO-Klinik Essen.
HNO. 1995 May;43(5):318-20.
Amyloidosis is a term that describes a heterogeneous group of disorders in which various tissues contain the characteristic insoluble fibrillar protein known as amyloid. A number of different forms of presentation have been described and include senile, familial or hereditary, systemic, localized, secondary and primary forms. We now report our management of a 79-year old woman who suffered from primary A-lambda-light chain amyloidosis. Amyloid changes first presented in the tissues of the tongue and floor of mouth and were seen clinically as a macroglossia. Further investigation led to a primary diagnosis of multiple myeloma IIa, as categorized by Durie and Salmon. Several cases have been reported to date describing secondary development of macroglossia due to long-term known and treated multiple myeloma. However, there has been only one report of macroglossia as a primary finding leading to the diagnosis of multiple myeloma.
淀粉样变性是一个术语，用于描述一组异质性疾病，其中各种组织含有被称为淀粉样蛋白的特征性不溶性纤维状蛋白质。已经描述了多种不同的表现形式，包括老年型、家族性或遗传性、全身性、局限性、继发性和原发性形式。我们现在报告我们对一名79岁患有原发性A-λ轻链淀粉样变性女性的治疗情况。淀粉样变首先出现在舌组织和口腔底部，临床上表现为巨舌症。进一步检查导致初步诊断为Durie和Salmon分类的IIa期多发性骨髓瘤。迄今为止，已有数例报告描述了由于长期已知并接受治疗的多发性骨髓瘤导致巨舌症的继发性发展。然而，仅有一份报告将巨舌症作为导致多发性骨髓瘤诊断的主要发现。
