PDF(Pubmed)
Abstract：
UNASSIGNED: Light-chain deposition disease (LCDD) is a systemic disorder characterized by non-amyloidotic light-chain deposition in various organs with Bence-Jones type monoclonal gammopathy. Although known as monoclonal gammopathy of renal significance, it may involve interstitial tissue of various organs, and in rare cases, proceeds to organ failure. We present a case of cardiac LCDD in a patient initially suspected of dialysis-associated cardiomyopathy.
UNASSIGNED: A 65-year-old man with end-stage renal disease requiring haemodialysis presented with fatigue, anorexia, and shortness of breath. He had a history of recurrent congestive heart failure and Bence-Jones type monoclonal gammopathy. A cardiac biopsy performed for suspected light-chain cardiac amyloidosis was negative for diagnostic Congo-red stain, however, paraffin immunofluorescence examination for light-chain suggested diagnosis of cardiac LCDD.
UNASSIGNED: Cardiac LCDD may go undetected leading to heart failure due to lack of clinical awareness and insufficient pathological investigation. In heart failure cases with Bence-Jones type monoclonal gammopathy, clinicians should consider not only amyloidosis but also interstitial light-chain deposition. In addition, in patients with chronic kidney disease of unknown cause, investigation is recommended to rule out cardiac light-chain deposition disease concomitant with renal LCDD. Although LCDD is relatively rare it occasionally affects multiple organs; therefore, it would be better to describe it as a monoclonal gammopathy of clinical significance rather than one of renal significance.
UNASSIGNED: A 65-year-old man with end-stage renal disease requiring haemodialysis presented with fatigue, anorexia, and shortness of breath. He had a history of recurrent congestive heart failure and Bence-Jones type monoclonal gammopathy. A cardiac biopsy performed for suspected light-chain cardiac amyloidosis was negative for diagnostic Congo-red stain, however, paraffin immunofluorescence examination for light-chain suggested diagnosis of cardiac LCDD.
UNASSIGNED: Cardiac LCDD may go undetected leading to heart failure due to lack of clinical awareness and insufficient pathological investigation. In heart failure cases with Bence-Jones type monoclonal gammopathy, clinicians should consider not only amyloidosis but also interstitial light-chain deposition. In addition, in patients with chronic kidney disease of unknown cause, investigation is recommended to rule out cardiac light-chain deposition disease concomitant with renal LCDD. Although LCDD is relatively rare it occasionally affects multiple organs; therefore, it would be better to describe it as a monoclonal gammopathy of clinical significance rather than one of renal significance.
摘要：
未经证实：轻链沉积病（LCDD）是一种全身性疾病，其特征是在Bence-Jones型单克隆丙种球蛋白病的各种器官中存在非淀粉样变性轻链沉积。尽管被称为具有肾脏意义的单克隆丙种球蛋白病，它可能涉及各种器官的间质组织，在极少数情况下，导致器官衰竭。我们介绍了最初怀疑透析相关心肌病的患者的心脏LCDD病例。
未经证实：一名65岁的终末期肾病患者，需要血液透析，表现为疲劳，厌食症,呼吸急促.他有复发性充血性心力衰竭和Bence-Jones型单克隆丙种球蛋白病的病史。对疑似轻链心脏淀粉样变性进行的心脏活检对诊断刚果红染色呈阴性，然而,轻链石蜡免疫荧光检查提示诊断为心脏LCDD。
未经证实：由于缺乏临床意识和病理检查不足，心脏LCDD可能未被发现，导致心力衰竭。在患有Bence-Jones型单克隆丙种球蛋白病的心力衰竭病例中，临床医生不仅要考虑淀粉样变性，还要考虑间质轻链沉积。此外,原因不明的慢性肾病患者，建议进行调查以排除心脏轻链沉积疾病并伴有肾脏LCDD.尽管LCDD相对罕见，但偶尔会影响多个器官；因此，最好将其描述为具有临床意义的单克隆丙种球蛋白病，而不是肾脏意义之一。
未经证实：一名65岁的终末期肾病患者，需要血液透析，表现为疲劳，
未经证实：由于缺乏临床意识和病理检查不足，心脏LCDD可能未被发现，导致心力衰竭。
