Isolated cardiovascular involvement in light chain amyloidosis

BMJ Case Rep. 2020 Apr 6;13(4):e233227. doi: 10.1136/bcr-2019-233227.


A 50-year-old woman presented with complaints of palpitations and breathlessness of 6 months' duration. She was being treated elsewhere as a case of dilated cardiomyopathy. On evaluation she had racoon eyes, poor progression of R wave on ECG and low-voltage complexes in the limb leads. Echocardiography revealed biventricular hypertrophy, diastolic dysfunction and moderate systolic dysfunction. Cardiac MRI showed features suggestive of amyloidosis. Bone marrow biopsy revealed raised plasma cell count, and endomyocardial biopsy showed amyloid deposits in the myocardium. Free lambda light chain levels were elevated, even though serum and urine electrophoresis did not show any monoclonal band. In this 'text book case of cardiac amyloidosis', apart from cardiovascular system no other organ system was affected, which is uncommon in primary light chain amyloidosis. The patient was started on CyBorD (cyclophosphamide, bortezomib and dexamethasone) regimen.

Keywords: cardiovascular medicine; heart failure.

Publication types

  • Case Reports
  • Video-Audio Media

MeSH terms

  • Amyloidosis / diagnosis*
  • Amyloidosis / drug therapy
  • Amyloidosis / pathology
  • Antineoplastic Agents / therapeutic use
  • Biopsy
  • Bone Marrow / pathology
  • Bortezomib / therapeutic use
  • Cardiomyopathy, Dilated / diagnosis*
  • Cardiomyopathy, Dilated / physiopathology
  • Cyclophosphamide / therapeutic use
  • Dexamethasone / therapeutic use
  • Drug Therapy, Combination
  • Dyspnea / etiology*
  • Echocardiography / methods
  • Electrocardiography / methods
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulin lambda-Chains / blood
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging / methods
  • Middle Aged
  • Myocardium / pathology
  • Treatment Outcome


  • Antineoplastic Agents
  • Glucocorticoids
  • Immunoglobulin lambda-Chains
  • Immunosuppressive Agents
  • Bortezomib
  • Dexamethasone
  • Cyclophosphamide