Legatowicz-Koprowska Marta, Szczygieł Justyna, Mańczak Małgorzata, Walczak Ewa
Department of Pathology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
Department of Cardiomyopathy, Cardinal Wyszynski National Institute of Cardiology, Warsaw, Poland.
Reumatologia. 2024;62(5):338-345. doi: 10.5114/reum/192390. Epub 2024 Nov 6.
Amyloidosis is a heterogeneous group of conditions associated with tissue deposition of insoluble abnormal proteins that damage vital organs. Early diagnosis, when the deposits are minimal, determines the prognosis and requires histological confirmation. The commonly adopted gold standard technique is alkaline Congo red (ACR) staining, though its sensitivity is limited. There is a need for a simple and inexpensive screening method offering a better chance of detecting minimal amyloid deposits. The aim of this study was to compare amyloid detectability with ACR and phenol Congo red (PHCR) staining techniques for early detection of minimal deposits.
We assessed 452 tissue specimens (including adipose tissue, gastrointestinal mucosa, labial salivary gland, myocardium, and bone marrow) from 425 patients with clinically suspected systemic or local amyloidosis, which had been sent to the Pathology Laboratory of the National Institute of Geriatrics, Rheumatology and Rehabilitation in Warsaw. Adjacent sections from each specimen were stained with ACR and PHCR. If amyloid was detected, immunohistochemical typing was conducted. The consistency of the two staining methods was expressed as Cohen's κ coefficient.
A total of 169 tissue specimens (37%) yielded positive readings, with 93 cases ACR(+) and PHCR(+); 75 cases ACR(-) and PHCR(+), and 1 case ACR(+) and PHCR(-). The percentage agreement between the staining methods was 83%, with the Cohen's κ coefficient value of 0.60 (95% CI: 0.52-0.69), which corresponds to moderate agreement according to Fleiss. Additional immunohistochemical amyloid typing, conducted in ACR(-) and PHCR(+) specimens, yielded conclusive results in 82% of cases.
The use of PHCR staining as a screening method in suspected amyloidosis improves amyloid (light chain, transthyretin, and amyloid A protein) detectability in various tissues. The PHCR staining specificity should be verified via electron microscopy and/or mass spectrometry.
淀粉样变性是一组异质性疾病,与不溶性异常蛋白在组织中的沉积有关,这些蛋白会损害重要器官。在沉积物极少时进行早期诊断可决定预后,且需要组织学证实。常用的金标准技术是碱性刚果红(ACR)染色,但其敏感性有限。需要一种简单且廉价的筛查方法,以便有更好的机会检测到极少的淀粉样沉积物。本研究的目的是比较ACR和苯酚刚果红(PHCR)染色技术在早期检测极少沉积物方面对淀粉样物质的可检测性。
我们评估了来自425例临床怀疑患有系统性或局部淀粉样变性患者的452份组织标本(包括脂肪组织、胃肠道黏膜、唇唾液腺、心肌和骨髓),这些标本已被送至华沙国家老年病学、风湿病学和康复研究所病理实验室。每个标本的相邻切片用ACR和PHCR染色。如果检测到淀粉样物质,则进行免疫组织化学分型。两种染色方法的一致性用科恩κ系数表示。
总共169份组织标本(37%)检测结果为阳性,其中93例ACR(+)且PHCR(+);75例ACR(-)且PHCR(+),1例ACR(+)且PHCR(-)。染色方法之间的百分比一致性为83%,科恩κ系数值为0.60(95%CI:0.52 - 0.69),根据弗莱伊斯的标准,这对应于中度一致性。在ACR(-)且PHCR(+)的标本中进行的额外免疫组织化学淀粉样物质分型,82%的病例得出了确定性结果。
在疑似淀粉样变性中使用PHCR染色作为筛查方法可提高各种组织中淀粉样物质(轻链、转甲状腺素蛋白和淀粉样A蛋白)的可检测性。PHCR染色的特异性应通过电子显微镜和/或质谱法进行验证。