Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2022; 10(34): 12703-12710
Published online Dec 6, 2022. doi: 10.12998/wjcc.v10.i34.12703
Pomolidomide for relapsed/refractory light chain amyloidosis after resistance to both bortezomib and daratumumab: A case report
Xian Li, Xiao-Hong Pan, Qiu Fang, Yun Liang
Xian Li, Yun Liang, Department of Hematology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China
Xiao-Hong Pan, Department of Cardiology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China
Qiu Fang, Department of Hematology, Huzhou Central Hospital, Affiliated Huzhou Hospital, Zhejiang University School of Medicine, Huzhou 313099, Zhejiang Province, China
Author contributions: Li X, Pan XH, and Liang Y contributed to the design and conception of the study; Li X and Fang Q contributed to data collection; Li X contributed to writing the initial drafting of the manuscript; Liang Y reviewed and edited the original draft; All authors contributed to manuscript revision and read and approved the submitted version.
Informed consent statement: All study participants or their legal guardian provided informed written consent about personal and medical data collection prior to study enrolment.
Conflict-of-interest statement: All the authors report having no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yun Liang, MD, Chief Physician, Department of Hematology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, No. 88 Jiefang Road, Hangzhou 310009, Zhejiang Province, China. liangyun@zju.edu.cn
Received: August 9, 2022
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
Abstract
BACKGROUND

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.

CASE SUMMARY

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.

CONCLUSION

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.

Keywords: Immunoglobulin light chain amyloidosis, Relapsed/refractory, Pleural effusion, Endo-myocardial biopsy, Immunomodulatory agent, Case report

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.