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Heavy and light chain (AHL)-type cardiac amyloidosis: first histopathologic-proven case illustrating involvement of the heart.
Virchows Archiv ( IF 3.4 ) Pub Date : 2020-05-09 , DOI: 10.1007/s00428-020-02837-1
Miroslav Sekulic 1 , Simona Pichler Sekulic 1 , Astrid Weins 2
Affiliation  

Cardiac amyloidosis is most commonly comprised of either a monoclonal immunoglobulin or transthyretin; however, in practice, detailing of the former beyond light chain restriction is not typically performed. We present briefly the case of an 80-year-old man with concern for cardiac amyloidosis and a subsequent endomyocardial biopsy revealing significant deposition of amorphous Congo red-positive material. By immunofluorescence microscopy, the amyloidogenic material showed positive expression for IgG heavy chain and kappa light chain, with negative staining for IgM and IgA heavy chains and lambda light chain supporting a diagnosis of heavy and light chain (AHL)-type amyloidosis. Immunofluorescence staining for the IgG heavy chain subclasses supported and further classified the patient's AHL-type cardiac amyloidosis as being IgG4/kappa restricted. The presented case is the first to illustrate AHL-type cardiac amyloidosis via sampling of heart tissue.

中文翻译:

重链和轻链(AHL)型心脏淀粉样变性:经组织病理学证实的首例病例说明心脏受累。

心脏淀粉样变性病最常见的是单克隆免疫球蛋白或运甲状腺素蛋白。然而,实际上,通常不进行轻链限制以外的前者的详细说明。我们简要介绍了一个有关心脏淀粉样变性和随后的心内膜活组织检查的80岁男性的病例,该病例显示无定形刚果红阳性材料的大量沉积。通过免疫荧光显微镜检查,淀粉样蛋白生成物质对IgG重链和κ轻链显示阳性表达,对IgM和IgA重链和lambda轻链染色阴性,支持诊断重链和轻链(AHL)型淀粉样变性。IgG重链亚类的免疫荧光染色支持并进一步分类了患者的 s AHL型心脏淀粉样变性受IgG4 / kappa限制。提出的案例是第一个通过心脏组织采样说明AHL型心脏淀粉样变性的案例。
更新日期:2020-05-09
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