# 淀粉样物质(Amyloid) - 器官 / 系统:肾上腺 / 内分泌系统(adrenal-gland / endocrine-system) - NTP 数据编号:ntp-0074 - 规范 URL:https://www.labsmart.cn/ntp/adrenal-gland-amyloid.html - 中文图谱总页锚点:https://www.labsmart.cn/ntp-atlas.html#adrenal-gland-amyloid - 原始出处:https://ntp.niehs.nih.gov/atlas/nnl/endocrine-system/adrenal-gland/Amyloid - 授权:美国联邦政府作品,公有领域;引用请标注 NTP Nonneoplastic Lesion Atlas - 适用说明:仅供科研使用,不用于临床诊断 ## 一句话结论 “淀粉样物质(amyloid)”一词经典上指多种不溶性、纤维状蛋白质,它们具有相似的构型(β-折叠片层)。 ## 病变描述(中文) “淀粉样物质(amyloid)”一词经典上指多种不溶性、纤维状蛋白质,它们具有相似的构型(β-折叠片层)。这些异常折叠的蛋白质在许多组织中形成并在细胞内和细胞外蓄积,原因众多,如遗传易感性以及各种病因引起的局部和全身性炎症。在大鼠中,局灶性(focal)或全身性淀粉样物质沉积很少发生于任何组织,包括肾上腺。在小鼠中,肾上腺及其他组织的淀粉样物质沉积总体上远为常见。发病率存在遗传相关性差异,某些品系如B6C3F1/N小鼠发病率很低,而其他品系如(Swiss)CD-1和Swiss Webster小鼠发病率则高得多。在小鼠中,淀粉样变性通常是一种自发性、与年龄相关的全身性疾病,肾上腺是较常受累的组织之一。严重程度(淀粉样物质/组织的量)在所有组织中,包括肾上腺,也倾向于随年龄增长而增加。在小鼠中,肾上腺淀粉样物质沉积通常首先表现为内皮质细胞之间的小团块,并呈进行性向心性扩展至外皮质(束状带)(Figure 1、Figure 2、Figure 3、Figure 4、Figure 5、Figure 6)。淀粉样蛋白质可形成大的、膨胀性聚集体(Figure 1、Figure 2),使正常结构变形,并最终可融合成环绕髓质的厚环状带(Figure 3、Figure 4)。淀粉样物质可延伸至髓质或球状带,但这些区域一般不受累。经苏木精和伊红(H&E)染色,淀粉样物质沉积物表现为均质、无定形、淡嗜酸性物质(Figure 1、Figure 2、Figure 3、Figure 4)。特殊组织化学染色常用于将淀粉样物质与纤维结缔组织、纤维蛋白及其他物质相区分。最广泛使用的染色之一为刚果红(Congo red)。经刚果红染色的淀粉样物质在非偏振光下呈淡至暗红色(Figure 5),但在偏振光下表现出明亮的双折射(通常为“苹果绿”)(Figure 6)。其他淀粉样物质检测方法包括荧光或异染性染色以及免疫组织化学。 ## 诊断与分级建议(NTP 共识,中文) 肾上腺中的淀粉样物质应诊断为存在,但不赋予严重程度分级。任何相关的细胞丢失或变性不应单独诊断,而应在病理学描述中加以描述。肾上腺淀粉样物质沉积是局限性的还是全身性分布的一部分,也应在描述中说明。如果双侧肾上腺均可见淀粉样物质,应在诊断中添加修饰词“双侧(bilateral)”(除非另有说明,病变假定为单侧)。 ## 英文原文 · Comment The term "amyloid" classically denotes various insoluble, fibrillar proteins that share a similar configuration (β-pleated sheets). These abnormally folded proteins form and accumulate intra- and extracellularly in many tissues due to many causes, such as genetic predisposition and local and systemic inflammation of various etiologies. In rats, focal or generalized amyloid deposition rarely occurs in any tissue, including the adrenal gland. In mice, amyloid deposition in the adrenal gland and other tissues is overall far more common. There are genetically related differences in incidence, with very low incidences in some strains, such as the B6C3F1/N mouse, and much higher incidences in other strains, such as (Swiss) CD-1 and Swiss Webster mice. In mice, amyloidosis is usually a spontaneous, age-related systemic disease, with the adrenal gland one of the more commonly affected tissues. Severity (amount of amyloid/tissue) also tends to increase with age in all tissues, including the adrenal gland. In mice, adrenal amyloid deposition usually first appears as small clumps between cells of the inner cortex, with progressive centripetal extension into the outer cortex (zona fasciculata) ( Figure 1 , Figure 2 , Figure 3 , Figure 4 , Figure 5 , and Figure 6 ). The amyloid proteins can form large, expansile aggregates ( Figure 1 and Figure 2 ), which distort the normal architecture and can eventually coalesce into thick circumferential bands that surround the medulla ( Figure 3 and Figure 4 ). The amyloid may extend into the medulla or zona glomerulosa, but these regions are generally unaffected. With hematoxylin and eosin (H&E) stain, amyloid deposits appear as homogeneous, amorphous, pale eosinophilic material ( Figure 1 , Figure 2 , Figure 3 , and Figure 4 ). Special histochemical stains are often used to distinguish amyloid from fibrous connective tissue, fibrin, and other substances. One of the most widely used stains is Congo red. Amyloid stained with Congo red appears pale to dull red under nonpolarized light ( Figure 5 ) but exhibits brilliant birefringence (usually "apple green") under polarized light ( Figure 6 ). Other methods for amyloid detection include fluorescent or metachromatic stains and immunohistochemistry. ## 英文原文 · Recommendation Amyloid in the adrenal should be diagnosed as present without assignment of a severity grade. Any associated loss or degeneration of cells should not be diagnosed separately but should be described in the pathology narrative. Whether the adrenal amyloid deposits are localized or part of a systemic distribution should also be addressed in the narrative. If amyloid is seen in both adrenal glands, the modifier "bilateral" should be added to the diagnosis (lesions are assumed to be unilateral unless otherwise indicated). ## 配图 - https://www.labsmart.cn/ntp-atlas-images/ntp_0219.jpg - https://www.labsmart.cn/ntp-atlas-images/ntp_0218.jpg 整理:北京天融慧通科技有限责任公司(LabSmart)| https://www.labsmart.cn