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表现为心房肿块的轻链沉积病:一例病例报告及文献复习

Light chain deposition disease presenting as an atrial mass: a case report and review of literature.

作者信息

Hudak Madeline, Sardana Ruhani, Parwani Anil V, Mathewson Roger C, Gibson Christopher G, Cohen P Aryeh, Lazarus John J, Bruce Jarrod T, Son Jae H, Tynski Zofia

机构信息

Department of Internal Medicine, Fairfield Medical Center, Lancaster, Ohio.

Department of Pathology, The Ohio State University Medical Center and Comprehensive Cancer Center, Columbus, Ohio.

出版信息

Cardiovasc Pathol. 2021 Nov-Dec;55:107368. doi: 10.1016/j.carpath.2021.107368. Epub 2021 Jul 27.

Abstract

Light chain deposition disease (LCDD) also known as nonamyloidotic immunoglobulin deposition disease is a rare systemic disorder due to the abnormal deposition of immunoglobulin in multiple organs caused by the clonal proliferation of B lymphocytes and plasma cells. Renal involvement is the most common with cardiac manifestations being the most common extra renal presentation of the disease. Renal involvement is not always associated with LCDD. Isolated cardiac involvement can manifest in a wide variety of ways: heart failure, cardiomyopathy, arrhythmias, angina, myocardial infarction, etc. We hereby present an unusual case of 59-year-old female who presented to clinic for routine follow up. A murmur on physical exam was evaluated with echocardiogram which led to the discovery of an incidental right atrial mass. Cardiac magnetic resonance imaging was completed 6 months later for follow up which showed increasing size of the mass. The mass was excised and found to be consistent with LCDD. To the best of our knowledge, this is the first reported case of LCDD manifesting as an atrial mass. Through this case report and review of literature we would like to generate awareness among our fellow pathologists and clinicians to maintain a high level of suspicion for LCDD as it can manifest in many unusual ways, with or without kidney involvement.

摘要

轻链沉积病(LCDD)也称为非淀粉样免疫球蛋白沉积病,是一种罕见的系统性疾病,由B淋巴细胞和浆细胞的克隆增殖导致免疫球蛋白在多个器官异常沉积引起。肾脏受累最为常见,心脏表现是该疾病最常见的肾外表现。肾脏受累并不总是与LCDD相关。孤立性心脏受累可表现为多种形式:心力衰竭、心肌病、心律失常、心绞痛、心肌梗死等。我们在此报告一例59岁女性的不寻常病例,该患者因常规随访前来就诊。体格检查时发现杂音,经超声心动图评估后发现了一个偶然发现的右心房肿块。6个月后进行心脏磁共振成像随访,结果显示肿块增大。肿块切除后发现与LCDD相符。据我们所知,这是首例报道的以心房肿块形式表现的LCDD病例。通过本病例报告和文献回顾,我们希望提高病理学家和临床医生同行的认识,因为LCDD可表现为许多不寻常的形式,无论有无肾脏受累,都要对其保持高度怀疑。

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