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限制性心肌病伴心肌κ轻链沉积作为多发性骨髓瘤的并发症。组织化学和电子显微镜观察。

Restrictive cardiomyopathy with kappa light chain deposits in myocardium as a complication of multiple myeloma. Histochemical and electron microscopic observations.

作者信息

McAllister H A, Seger J, Bossart M, Ferrans V J

机构信息

Department of Pathology, St. Luke's Episcopal Hospital, Houston, TX 77030.

出版信息

Arch Pathol Lab Med. 1988 Nov;112(11):1151-4.

PMID:3140760
Abstract

kappa Light chain deposits occurring in myocardium as a complication of multiple myeloma were identified ultrastructurally and immunohistochemically in a right ventricular endomyocardial biopsy specimen from a patient who presented with clinical and hemodynamic findings of restrictive cardiomyopathy. These deposits were not evident on routine histopathologic examination; they were Congo red-negative and gave a positive immunoperoxidase reaction for kappa light chains and a negative reaction for lambda chains. They consisted of amorphous, electron-dense granules that formed discontinuous layers adjacent to the plasma membranes of cardiac myocytes, arteriolar endothelial and smooth-muscle cells, and neural elements. These observations underscore the need for critical study of endomyocardial biopsy specimens, using electron microscopy and immunohistochemical reagents, for the precise identification of protein components in tissue deposits in patients suspected of having cardiac amyloidosis or related disorders.

摘要

在一名出现限制性心肌病临床和血流动力学表现的患者的右心室心内膜活检标本中,通过超微结构和免疫组织化学方法鉴定出κ轻链沉积物,这是多发性骨髓瘤的一种并发症。这些沉积物在常规组织病理学检查中不明显;它们刚果红染色阴性,κ轻链免疫过氧化物酶反应阳性,λ链反应阴性。它们由无定形、电子致密颗粒组成,这些颗粒在心肌细胞、小动脉内皮和平滑肌细胞以及神经成分的质膜附近形成不连续层。这些观察结果强调,对于疑似患有心脏淀粉样变性或相关疾病的患者,需要使用电子显微镜和免疫组织化学试剂对心内膜活检标本进行严格研究,以精确鉴定组织沉积物中的蛋白质成分。

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