PMID- 34692719 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211026 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 8 DP - 2021 TI - Case Report: Lenalidomide as a Second-Line Treatment for Bortezomib-Ineffective Nephrotic Syndrome Caused by LCDD: 2 Case Reports and a Literature Review. PG - 706971 LID - 10.3389/fmed.2021.706971 [doi] LID - 706971 AB - Background: Light-chain deposition disease (LCDD) is a rare systemic disorder characterized by the deposition of monoclonal light chains in organs. The kidney is a prominent target of light-chain deposition, with a median time to end-stage renal disease (ESRD) of 2.7 years and 5-year ESRD-free survival of 37%. The therapeutic management of LCDD remains ill-defined. In addition to bortezomib-based therapy as first-line therapy, the effect of lenalidomide on LCDD is rarely reported. Case Presentation: This study describes two male LCDD patients in their 60s with nephrotic syndrome and moderately impaired renal function. One patient had monoclonal IgGlambda with underlying MGRS, and another had monoclonal IgGkappa with underlying monoclonal gammopathy that developed into symptomatic MM during follow-up. The hallmarks of this disease were consistent with previous reports. Both patients initially received BCD therapy, but no hematological response was observed. Consequently, the nephrotic syndrome was refractory. Sequential Rd therapy was initiated, and partial hematological response and nephrotic remission were observed in the IgGlambda patient but absent in the IgGkappa patient. Conclusion: Limited reports have demonstrated the effect of lenalidomide in LCDD. We report the outcome of lenalidomide in two cases of bortezomib-resistant LCDD. This treatment might be a beneficial supplement for those unresponsive or intolerant to bortezomib in LCDD, but the effect should be prospectively investigated. CI - Copyright (c) 2021 Zhang, Yu, Wang, Zhou and Zhao. FAU - Zhang, Xin AU - Zhang X AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. FAU - Yu, Xiao-Juan AU - Yu XJ AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Renal Pathology Center, Institute of Nephrology, Peking University First Hospital, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, China. AD - Research Units of Diagnosis and Treatment of Immune-Mediated Kidney Diseases, Chinese Academy of Medical Sciences, Beijing, China. FAU - Wang, Su-Xia AU - Wang SX AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Renal Pathology Center, Institute of Nephrology, Peking University First Hospital, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, China. AD - Research Units of Diagnosis and Treatment of Immune-Mediated Kidney Diseases, Chinese Academy of Medical Sciences, Beijing, China. AD - Laboratory of Electron Microscopy, Pathological Centre, Peking University First Hospital, Beijing, China. FAU - Zhou, Fu-de AU - Zhou FD AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Renal Pathology Center, Institute of Nephrology, Peking University First Hospital, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, China. AD - Research Units of Diagnosis and Treatment of Immune-Mediated Kidney Diseases, Chinese Academy of Medical Sciences, Beijing, China. FAU - Zhao, Ming-Hui AU - Zhao MH AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Renal Pathology Center, Institute of Nephrology, Peking University First Hospital, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Ministry of Education of China, Beijing, China. AD - Research Units of Diagnosis and Treatment of Immune-Mediated Kidney Diseases, Chinese Academy of Medical Sciences, Beijing, China. AD - Laboratory of Electron Microscopy, Pathological Centre, Peking University First Hospital, Beijing, China. AD - Peking-Tsinghua Center for Life Science, Beijing, China. LA - eng PT - Case Reports DEP - 20211008 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC8531272 OTO - NOTNLM OT - case report OT - lenalidomide OT - light-chain deposition disease OT - monoclonal gammopathies of renal significance OT - nephrotic syndrome (NS) COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2021/10/26 06:00 MHDA- 2021/10/26 06:01 PMCR- 2021/10/08 CRDT- 2021/10/25 06:39 PHST- 2021/05/08 00:00 [received] PHST- 2021/09/13 00:00 [accepted] PHST- 2021/10/25 06:39 [entrez] PHST- 2021/10/26 06:00 [pubmed] PHST- 2021/10/26 06:01 [medline] PHST- 2021/10/08 00:00 [pmc-release] AID - 10.3389/fmed.2021.706971 [doi] PST - epublish SO - Front Med (Lausanne). 2021 Oct 8;8:706971. doi: 10.3389/fmed.2021.706971. eCollection 2021.