Cardiac Tamponade From Lymphocytic Constrictive Pericarditis in Primary AL Amyloidosis Without Myocardial Involvement
4 affiliations
- 1Department of Cardiology, Changi General Hospital, Singapore
- 2Department of Infectious Diseases, Changi General Hospital, Singapore
- 3Department of Pathology, Singapore General Hospital, Singapore
- 4Department of Hematology, Singapore General Hospital, Singapore
Abstract
Background: Isolated pericardial involvement in systemic light chain (AL) amyloidosis without myocardial infiltration is extraordinarily rare. Constrictive pericarditis with cardiac tamponade as the sole cardiac manifestation has not been previously reported.
Case Summary: A 69-year-old man presented with exertional dyspnea and was diagnosed with a large pericardial effusion with features of constrictive pericarditis. Pericardiocentesis and pericardial biopsy showed lymphocytic effusion without cardiac amyloid involvement. Biopsy of a prevertebral mass later revealed amyloid deposition, confirming systemic AL amyloidosis. He was treated with immunochemotherapy but was unfit for autologous stem cell transplant.
Discussion: This case highlights the diagnostic challenges of AL amyloidosis, especially with concomitant pericardial disease without myocardial involvement. Pericardial disease may reflect an inflammatory process rather than direct amyloid infiltration. Diagnosis is challenging given atypical presentations and limited sensitivities of initial monoclonal protein testing.
Take-Home Messages: Consider AL amyloidosis in unexplained pericardial disease. Negative monoclonal studies do not exclude systemic amyloidosis. Pursue myocardial biopsy if suspicion persists.
Related
- Gastric involvement in multiple myeloma-associated AL amyloidosis: a case with serial endoscopy and a pooled analysis of 30 published cases.
- Advanced Echocardiography and Myocardial Work in Idiopathic Carpal Tunnel Syndrome with Presumptive Concurrent Cardiac Amyloidosis: An Exploratory Cross-Sectional Study.
- Myocardial work in light-chain and transthyretin-related amyloidosis with preserved ejection fraction: a cardiovascular magnetic resonance study
- A Progressive 99mTc-MDP Uptake in Transthyretin Cardiac Amyloidosis
- The Progressive Cardiac Uptake of 99mTc-MDP as a Clue to Transthyretin Amyloidosis
BibTeX
@article{leong2026cardiac,
title = {{Cardiac Tamponade From Lymphocytic Constrictive Pericarditis in Primary AL Amyloidosis Without Myocardial Involvement}},
author = {Yen Hsin Leong and Zhenwei Teo and Edwin Chong Yu Sng and Siang Hui Lai and Marvin Raden Torres De Guzman and Felix Maverick Uy},
journal = {JACC Case Reports},
year = {2026},
month = aug,
doi = {10.1016/j.jaccas.2026.109538},
url = {https://doi.org/10.1016/j.jaccas.2026.109538},
note = {CC BY-NC-ND 4.0},
}
RIS
TY - JOUR
TI - Cardiac Tamponade From Lymphocytic Constrictive Pericarditis in Primary AL Amyloidosis Without Myocardial Involvement
AU - Yen Hsin Leong
AU - Zhenwei Teo
AU - Edwin Chong Yu Sng
AU - Siang Hui Lai
AU - Marvin Raden Torres De Guzman
AU - Felix Maverick Uy
PY - 2026
DA - 2026/08/06
JO - JACC Case Reports
DO - 10.1016/j.jaccas.2026.109538
UR - https://doi.org/10.1016/j.jaccas.2026.109538
AB - Background: Isolated pericardial involvement in systemic light chain (AL) amyloidosis without myocardial infiltration is extraordinarily rare. Constrictive pericarditis with cardiac tamponade as the sole cardiac manifestation has not been previously reported. Case Summary: A 69-year-old man presented with exertional dyspnea and was diagnosed with a large pericardial effusion with features of constrictive pericarditis. Pericardiocentesis and pericardial biopsy showed lymphocytic effusion without cardiac amyloid involvement. Biopsy of a prevertebral mass later revealed amyloid deposition, confirming systemic AL amyloidosis. He was treated with immunochemotherapy but was unfit for autologous stem cell transplant. Discussion: This case highlights the diagnostic challenges of AL amyloidosis, especially with concomitant pericardial disease without myocardial involvement. Pericardial disease may reflect an inflammatory process rather than direct amyloid infiltration. Diagnosis is challenging given atypical presentations and limited sensitivities of initial monoclonal protein testing. Take-Home Messages: Consider AL amyloidosis in unexplained pericardial disease. Negative monoclonal studies do not exclude systemic amyloidosis. Pursue myocardial biopsy if suspicion persists.
SN - 2666-0849
ER -