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限制性心肌病的心脏移植:既往单克隆丙种球蛋白病中心脏淀粉样变性的发展

Heart transplantation for restrictive cardiomyopathy: development of cardiac amyloidosis in preexisting monoclonal gammopathy.

作者信息

Deng M, Park J W, Roy-Chowdury R, Knieriem H J, Reinhard U, Heinrich K W

机构信息

Duisburg Heart Institute, Bethesda Hospital Duisburg, Germany.

出版信息

J Heart Lung Transplant. 1992 Jan-Feb;11(1 Pt 1):139-41.

PMID:1540601
Abstract

A 54-year-old woman died 6 months after heart transplantation for treatment of restrictive cardiomyopathy. A monoclonal gammopathy without other signs of malignant disease was present preoperatively, and up to 6 weeks before transplantation no evidence of amyloidosis was established in the rectal, bone marrow, and cardiac specimens. At autopsy there was amyloidosis type AL in the kidneys, bone marrow, liver, spleen, recipient atrium, and donor heart. Retrospectively, the explanted heart also revealed amyloidosis. We conclude that in patients undergoing heart transplantation for treatment of restrictive cardiomyopathy with a preexisting monoclonal gammopathy, a thorough evaluation, including multiorgan biopsy for amyloidosis with electron microscopic workup, should be performed.

摘要

一名54岁女性在接受心脏移植治疗限制性心肌病6个月后死亡。术前存在单克隆丙种球蛋白病但无其他恶性疾病迹象,直至移植前6周,直肠、骨髓和心脏标本均未发现淀粉样变性证据。尸检发现肾脏、骨髓、肝脏、脾脏、受者心房和供体心脏存在AL型淀粉样变性。回顾性分析,切除的心脏也显示有淀粉样变性。我们得出结论,对于因限制性心肌病接受心脏移植且术前已存在单克隆丙种球蛋白病的患者,应进行全面评估,包括多器官活检以诊断淀粉样变性并进行电子显微镜检查。

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