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日本单家机构尸检病例中心血管淀粉样变性的病理分析。

Pathological review of cardiac amyloidosis using autopsy cases in a single Japanese institution.

机构信息

Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka-shi, Fukuoka-ken 812-8582, Japan.

Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka-shi, Fukuoka-ken 812-8582, Japan.

出版信息

Pathol Res Pract. 2021 Nov;227:153635. doi: 10.1016/j.prp.2021.153635. Epub 2021 Sep 28.

Abstract

AIM

Amyloidosis is a systemic or localized disease of protein deposition characterized by amorphous eosinophilic morphology and positivity of Congo Red staining. The typing of amyloidosis is becoming increasingly important because therapeutic agents for each amyloidosis type have been developed. Herein, the authors review the autopsy cases at an institution to reveal the putative Japanese characteristics of each amyloidosis type and evaluate the clinicopathological significance of each type.

MATERIALS AND METHODS

A total of 131 autopsy cases of systemic and localized amyloidosis were retrieved for classification by immunohistochemistry. Immunohistochemistry for transthyretin, amyloid A (AA), immunoglobulin light-chain kappa and lambda, and β2-microglobulin was performed for all cases.

RESULTS

The 131 amyloidosis cases were classified as follows: 71 cases (54.2%) of transthyretin amyloidosis, 32 cases (24.4%) of AA amyloidosis, 8 cases (6.1%) of light-chain amyloidosis, and 5 cases (3.8%) of β2-microglobulin amyloidosis, along with 15 equivocal cases (11.5%). All cases showed myocardial involvement of amyloidosis. Histopathologically, the transthyretin type was significantly associated with the interstitial and nodular patterns, and with the absence of the perivascular and endocardial patterns. The AA type was significantly associated with the perivascular and endocardial patterns, and with the absence of the nodular pattern.

CONCLUSION

The authors revealed the putative characteristics of cardiac amyloidosis in Japan by using autopsy cases. About 90% of amyloidosis cases were successfully classified using only commercially available antibodies.

摘要

目的

淀粉样变性是一种以蛋白沉积为特征的系统性或局限性疾病,其形态学表现为无定形嗜酸性,刚果红染色阳性。淀粉样变性的分型变得越来越重要,因为已经开发出针对每种淀粉样变性类型的治疗药物。本文作者回顾了一家机构的尸检病例,以揭示每种淀粉样变性类型可能存在的日本特征,并评估每种类型的临床病理意义。

材料和方法

通过免疫组织化学方法对 131 例系统性和局限性淀粉样变性的尸检病例进行分类。对所有病例均进行转甲状腺素蛋白、淀粉样 A(AA)、免疫球蛋白轻链 κ 和 λ 以及β2-微球蛋白的免疫组织化学染色。

结果

这 131 例淀粉样变性病例的分类如下:71 例(54.2%)转甲状腺素蛋白淀粉样变性、32 例(24.4%)AA 淀粉样变性、8 例(6.1%)轻链淀粉样变性和 5 例(3.8%)β2-微球蛋白淀粉样变性,以及 15 例(11.5%)可疑病例。所有病例均有心肌淀粉样变性。组织病理学上,转甲状腺素蛋白型与间质和结节模式显著相关,与血管周围和心内膜模式无关。AA 型与血管周围和心内膜模式显著相关,与结节模式无关。

结论

作者通过尸检病例揭示了日本心脏淀粉样变性的可能特征。使用市售的抗体,约 90%的淀粉样变性病例可以成功分类。

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