Cardiac light-chain deposition disease and hints at diagnosing: a case report

Abstract

Background Light-chain deposition disease is a systemic disorder characterized by nonamyloidotic 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 light-chain deposition disease in a patient initially suspected of dialysis-associated cardiomyopathy. Case Summary A 65-year-old man with end-stage renal disease requiring hemodialysis 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 light-chain deposition disease. Discussion Cardiac light-chain deposition disease 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. Additionally, in patients with chronic kidney disease of unknown cause, investigation is recommended to rule out cardiac light-chain deposition disease concomitant with renal light-chain deposition disease. Although light-chain deposition disease 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.

Light chain deposition disease presenting with gastrointestinal disorder as primary manifestation: report of two cases and literature review

“…Although LCDD is a well-known monoclonal gammopathy of renal significance, cardiac 12 or gastrointestinal manifestations are also clinically very important. We analyzed our two patients based on the assessment of organ involvement in amyloid light chain amyloidosis.…”

Cardiomyopathy Without Amyloid Deposit in Systemic Light Chain Deposition Disease

“…Some cases suggest potential reversibility with plasma cell dyscrasia chemotherapy, while others result in fatal cardiac failure despite conservative treatment. 3) 4) The prognosis of LCDD varies, with around 32% of patients showing cardiac involvement and a poor prognosis. 5) This is the first reported case of LCDD with cardiac and renal involvement in Korea.…”