Matsuo Flávia Sayuri, Barbosa de Paulo Luiz Fernando, Servato João Paulo Silva, de Faria Paulo Rogério, Cardoso Sergio Vitorino, Loyola Adriano Mota
Universidade Federal de Uberlandia, Uberlandia, Minas Gerais, Brazil.
Clin Oral Investig. 2016 Nov;20(8):1913-1920. doi: 10.1007/s00784-015-1649-3. Epub 2015 Nov 10.
Amyloidosis is a term used to describe a group of diseases in which there is an extracellular deposition of amorphous fibrillar proteins known as amyloid. The aim of this study was to present clinicopathological data from eight oral amyloidosis-affected patients and a deep review of the literature about the disease.
A retrospective study was conducted based on the records of oral amyloidosis-affected patients diagnosed in our institution between 1978 and 2012. The clinicopathological features and immunohistochemical (IHC) staining with anti-kappa and anti-lambda light chain antibodies were carried out and analyzed.
Eight patients were diagnosed with the disease; the tongue and women in their sixth decade of life were mostly affected. All lesions demonstrated apple-green birefringence and immunoreactivity for kappa-light chain, and four cases also showed lambda positivity. According to our series, four cases were diagnosed with localized amyloidosis and four with systemic amyloidosis. Prognosis for the systemic ones was gloomy, but good for the localized ones, which was characterized by a slow pattern of deposition without evolution to systemic involvement.
This study reinforces our knowledge about predilections, outcomes, and the importance of making a correct and quick diagnosis of oral amyloidosis and shows the necessity of more studies detailing oral amyloidosis predilection on a global scale. The importance and utility of IHC in the typing of the biochemical nature of amyloid deposits are becoming increasingly necessary for proper management of the patient. Correct classification of the type of amyloid is important for treatment consequences.
This article highlights the clinicopathological data of patients with amyloidosis affecting oral tissues and compare these new findings with other worldwide descriptions. Because of its rarity, such data are often unfamiliar to most clinicians and pathologists.
淀粉样变性是一个用于描述一组疾病的术语,在这些疾病中存在称为淀粉样蛋白的无定形纤维状蛋白质的细胞外沉积。本研究的目的是呈现8例口腔淀粉样变性患者的临床病理数据,并对该疾病的文献进行深入回顾。
基于1978年至2012年在本机构诊断的口腔淀粉样变性患者的记录进行回顾性研究。进行并分析临床病理特征以及用抗κ和抗λ轻链抗体进行的免疫组织化学(IHC)染色。
8例患者被诊断患有该疾病;舌部以及60多岁的女性受影响最为常见。所有病变均显示苹果绿双折射以及κ轻链免疫反应性,4例还显示λ阳性。根据我们的系列病例,4例被诊断为局限性淀粉样变性,4例为系统性淀粉样变性。系统性淀粉样变性患者的预后不佳,但局限性淀粉样变性患者预后良好,其特征为沉积缓慢且无进展为系统性受累。
本研究强化了我们对口腔淀粉样变性的偏好、预后以及正确快速诊断的重要性的认识,并表明有必要开展更多研究以在全球范围内详细阐述口腔淀粉样变性的偏好。IHC在淀粉样沉积物生化性质分型中的重要性和实用性对于患者的恰当管理变得越来越必要。淀粉样变性类型的正确分类对于治疗结果很重要。
本文重点介绍了影响口腔组织的淀粉样变性患者的临床病理数据,并将这些新发现与全球其他描述进行比较。由于其罕见性,大多数临床医生和病理学家通常对这些数据并不熟悉。