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系统性轻链淀粉样变性骨髓涂片内的淀粉样物质

Amyloid in bone marrow smears in systemic light-chain amyloidosis.

作者信息

Kimmich Christoph, Schönland Stefan, Kräker Sandra, Andrulis Mindaugas, Ho Anthony D, Mayer Gudrun, Dittrich Tobias, Hundemer Michael, Hegenbart Ute

机构信息

a Amyloidosis Center, Department of Medicine V , University Hospital Heidelberg , Heidelberg , Germany.

b Department of General Pathology , University Hospital Ulm , Ulm , Germany.

出版信息

Amyloid. 2017 Mar;24(1):52-59. doi: 10.1080/13506129.2017.1314959. Epub 2017 Apr 23.

Abstract

We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR. No patient without systemic AL amyloidosis stained positive. Sensitivity for routine BM histology was 57% (74/129) and for fat aspiration 96% (134/140). Patients with amyloid on BM smears had significantly more hepatic (42 vs. 9%, p < .001), renal (78 vs. 43%, p < .001) and gastrointestinal involvement (40 vs. 22%, p < .01) and less commonly cardiac involvement (58 vs. 76%, p < .03) and consecutively no adverse prognosis. CR staining of BM smears cannot be recommended as a primary screening tool for systemic AL as its overall sensitivity is far inferior to BM histology and fat aspiration. However, we recommend using the technique when suspecting amyloid on Pappenheim staining to establish the diagnosis of systemic AL amyloidosis.

摘要

我们对系统性轻链(AL)淀粉样变性患者的骨髓(BM)涂片进行了前瞻性敏感性分析,这些涂片用刚果红(CR)染色并按照帕彭海姆法进行处理。将结果直接与常规BM组织学和脂肪抽吸结果进行比较。我们分析了198例诊断为或疑似系统性AL淀粉样变性患者的BM涂片。最终,168例患者得以确诊。在BM涂片上,CR检测到淀粉样物质的比例为33%(56/168)。所有在帕彭海姆染色上疑似淀粉样物质的患者(n = 39)在CR染色上均显示有大量淀粉样物质浸润。无系统性AL淀粉样变性的患者染色均为阴性。常规BM组织学的敏感性为57%(74/129),脂肪抽吸的敏感性为96%(134/140)。BM涂片上有淀粉样物质的患者肝脏受累(42%对9%,p <.001)、肾脏受累(78%对43%,p <.001)和胃肠道受累(40%对22%,p <.01)更为常见,而心脏受累较少见(58%对76%,p <.03),且无不良预后。不推荐将BM涂片的CR染色作为系统性AL的主要筛查工具,因为其总体敏感性远低于BM组织学和脂肪抽吸。然而,当在帕彭海姆染色上怀疑有淀粉样物质时,我们建议使用该技术来确诊系统性AL淀粉样变性。

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