CASE COMMUNICATIONS
IMAJ | volume 26
Journal 10, November 2024
pages: 660-662
Cardiac and Pleural Involvement in Systemic Light Chain Amyloidosis: A Case Report
1 Department of Cardiology, Meir Medical Center, Kfar Saba, Israel
2 Department of Pulmonology, Meir Medical Center, Kfar Saba, Israel
3 Department of Pathology, Meir Medical Center, Kfar Saba, Israel
4 Division of Hematology, Meir Medical Center, Kfar Saba, Israel
5 Department of Internal Medicine A, Meir Medical Center, Kfar Saba, Israel
6 Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Summary
Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting from the accumulation of misfolded protein aggregates in the myocardial extracellular space. Cardiac amyloidosis stems primarily from the misfolding of monoclonal immunoglobulin light chains (AL) originating from abnormal clonal plasma cell proliferation or transthyretin amyloidosis (ATTR).
Diagnosis of amyloidosis demands a high index of suspicion and requires histological confirmation of pathognomonic green birefringence under polarized light when an infiltrated tissue specimen is stained with Congo-red staining [1,2].
Pleural involvement of systemic amyloidosis has rarely been reported and is considered a serious complication [3]. Pleural amyloidosis is diagnosed with the identification of amyloid deposits in pleural biopsy specimens. However, pleural biopsies are rarely performed for this indication.
We describe the case of a patient with AL cardiac amyloidosis presenting as intractable pleural effusion and diagnosed via pleural biopsy.