Published online Dec 6, 2022. doi: 10.12998/wjcc.v10.i34.12703
Peer-review started: August 9, 2022
First decision: October 12, 2022
Revised: October 13, 2022
Accepted: November 4, 2022
Article in press: November 4, 2022
Published online: December 6, 2022
Processing time: 115 Days and 7.8 Hours
Immunoglobulin light chain (AL) amyloidosis is a rare disease characterized by deposition of ALs essentially in any organ or tissue, with cardiac involvement being very frequent (61%). Early diagnosis is of high importance because early initiation of treatment in AL amyloidosis may improve outcomes. Despite the administration of immunotherapeutic agents, in particular bortezomib and daratumumab, which have improved the outcomes of AL amyloidosis, anti-plasma cell therapy remains suboptimal for some patients.
We report the case of a 55-year-old man presenting with heart failure who was diagnosed with cardiac AL amyloidosis by an endomyocardial biopsy. He experienced a short-term hematological remission with no organ response after being administered a bortezomib-daratumumab containing regimen. The treatment was switched to pomolidomide due to pulmonary involvement and progressive pleural effusion, in which flow cytometry analysis showed abnormal plasma cells. After two cycles of this regimen, the pleural effusion was controlled effectively with no recurrence.
This case emphasizes the crucial role of endomyocardial biopsy in early diagnosis of cardiac amyloidosis and suggests that pomolidomide may be an effective treatment for patients with AL amyloidosis that is relapsed/refractory to both bortezomib and daratumumab.
Core Tip: We report the case of a 55-year-old man presenting with heart failure who was diagnosed with cardiac immunoglobulin light chain (AL) amyloidosis by an endomyocardial biopsy, in whom pomolidomide corrected severe pleural effusion after resistance to both bortezomib and daratumumab. This case emphasizes the crucial role of endomyocardial biopsy in early diagnosis of cardiac amyloidosis and suggests that pomolidomide may be an effective treatment for patients with AL amyloidosis that is relapsed/refractory to both bortezomib and daratumumab.