Krishnan J, Chu W S, Elrod J P, Frizzera G
Department of Hematologic and Lymphatic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.
Am J Clin Pathol. 1993 Aug;100(2):135-44. doi: 10.1093/ajcp/100.2.135.
Amyloidosis in its diverse types (immunocytic dyscrasia-associated, reactive, or heredofamilial) most often presents in a systemic form. Localized amyloidosis is uncommon in general and is exceedingly rare in the soft tissues. The authors discuss the cases of 14 patients in whom amyloidosis manifested as a localized mass ("amyloidoma") in the soft tissues (mostly mediastinal and retroperitoneal), leading to a clinical diagnosis of neoplasm in most cases. On the basis of the associated morphologically atypical and phenotypically monoclonal cell population, the resistance to potassium permanganate pretreatment, and the lack of reactivity with anti-AA antisera, 10 cases could be classified as immunocytic dyscrasia-associated AL-amyloidosis. However, four cases had histopathologic and histo- and immunohistochemical characteristics of reactive ("secondary") AA-amyloidosis. This proportion (28.5%) was higher than that suggested by the sporadic AA-amyloidomas reported in the literature. The pathologic distinction between these two categories is important because patients with AA-amyloidomas of the soft tissues appear to have a better prognosis.
淀粉样变性有多种类型（免疫细胞发育异常相关型、反应性或遗传家族型），大多以全身性形式出现。局限性淀粉样变性总体上并不常见，在软组织中极为罕见。作者讨论了14例淀粉样变性表现为软组织（主要是纵隔和腹膜后）局限性肿块（“淀粉样瘤”）的病例，多数病例临床诊断为肿瘤。基于相关的形态学非典型和表型单克隆细胞群、对高锰酸钾预处理的抗性以及与抗AA抗血清无反应性，10例可归类为免疫细胞发育异常相关的AL型淀粉样变性。然而，4例具有反应性（“继发性”）AA型淀粉样变性的组织病理学、组织学和免疫组织化学特征。这一比例（28.5%）高于文献报道的散发性AA型淀粉样瘤所提示的比例。这两类之间的病理区分很重要，因为软组织AA型淀粉样瘤患者的预后似乎较好。
