{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T04:57:26Z","timestamp":1781585846736,"version":"3.54.5"},"reference-count":36,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T00:00:00Z","timestamp":1781568000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>\n                    Waldenstr\u00f6m macroglobulinemia (WM) is a rare indolent B-cell lymphoma, characterized by lymphoplasmacytic infiltration of bone marrow and the existence of monoclonal IgM in circulation. Renal AL amyloidosis is an uncommon complication of WM, and its treatment is an enormous challenge to clinicians. This report describes a 71-year-old woman who presented with a 10-month history of recurrent bilateral lower extremity edema and a progressive fatigue. Physical and laboratory examinations revealed serum monoclonal IgM\u03bb, nephrotic syndrome, moderate anemia and mild enlargement of bilateral inguinal superficial lymph nodes. Bone marrow aspiration demonstrated clonal lymphoplasmacytic infiltration, lymph node biopsy was consistent with lymphoplasmacytic lymphoma, and peripheral blood testing confirmed the presence of the\n                    <jats:italic>MYD88<\/jats:italic>\n                    <jats:sup>L265P<\/jats:sup>\n                    mutation. Renal biopsy confirmed the diagnosis of renal AL amyloidosis, evidenced by Congo red-positive amorphous deposits with \u03bb light chain restriction in glomeruli, interstitium, and small arterial walls. Following multidisciplinary consultation, the patient was treated with the bendamustine plus rituximab (BR) regimen. After three cycles, the patient achieved partial hematologic response (serum IgM levels decreased from 23.7 g\/L to 7.1 g\/L) with slight improvement of proteinuria (urine protein excretion decreased from 18.2 g\/d to 15.0 g\/d). However, during the subsequent treatment, the patient developed a severe pulmonary infection that led to death. This case underscores two critical points: First, patients with WM should be routinely tested for serum and urine monoclonal free light chains, as they can exist in WM at a high proportion and can independently induce monoclonal gammopathy-associated renal diseases, including renal amyloidosis. Second, for WM patients with renal AL amyloidosis, choosing BR regimen as the first-line treatment is reasonable. However, during the treatment process, it is essential to closely monitor and actively prevent the adverse effects of this regimen, particularly severe infections which may have fatal consequences.\n                  <\/jats:p>","DOI":"10.3389\/fimmu.2026.1821051","type":"journal-article","created":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T04:29:50Z","timestamp":1781584190000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Waldenstr\u00f6m macroglobulinemia-associated renal AL amyloidosis: a case report and literature review"],"prefix":"10.3389","volume":"17","author":[{"given":"Jianping","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Nephrology, Xiamen Humanity Hospital, Fujian Medical University","place":["Xiamen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhifeng","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology, The First Affiliated Hospital of Xiamen University","place":["Xiamen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huiping","family":"Chen","sequence":"additional","affiliation":[{"name":"Expert Consultation Office, Jinling Hospital","place":["Nanjing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyan","family":"Bian","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Xiamen Humanity Hospital, Fujian Medical University","place":["Xiamen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Di","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Xiamen Humanity Hospital, Fujian Medical University","place":["Xiamen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhu","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Xiamen Humanity Hospital, Fujian Medical University","place":["Xiamen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiekun","family":"Li","sequence":"additional","affiliation":[{"name":"Nanjing Kingmed Diagnostics","place":["Nanjing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanlin","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Xiamen Humanity Hospital, Fujian Medical University","place":["Xiamen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,16]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01620-2","article-title":"The 5th edition of the World Health Organization classification of haematolymphoid tumours: Lymphoid neoplasms","volume":"36","author":"Alaggio","year":"2022","journal-title":"Leukemia"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022015851","article-title":"The International Consensus Classification of mature lymphoid neoplasms: A report from the Clinical Advisory Committee","volume":"140","author":"Campo","year":"2022","journal-title":"Blood"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.4084\/MJHID.2024.061","article-title":"Waldenstr\u00f6m macroglobulinemia - A state-of-the-art review: Part 1: Epidemiology, pathogenesis, clinicopathologic characteristics, differential diagnosis, risk stratification, and clinical problems","volume":"16","author":"Bibas","year":"2024","journal-title":"Mediterr J Hematol Infect Dis"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2024.105708","article-title":"Lymphoplasmacytic lymphoma and Waldenstr\u00f6m macroglobulinemia, a decade after the discovery of MYD88L265P","volume":"156","author":"Qiu","year":"2025","journal-title":"Hum Pathol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1080\/10428194.2025.2512392","article-title":"Not just amyloidosis: The spectrum of Waldenstrom's macroglobulinemia-associated renal disease","volume":"66","author":"Giachetti","year":"2025","journal-title":"Leuk Lymphoma"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1002\/cnr2.2062","article-title":"When Waldenstr\u00f6m macroglobulinemia hits the kidney: Description of a case series and management of a \"rare in rare\" scenario","volume":"7","author":"Danesin","year":"2024","journal-title":"Cancer Rep"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfy320","article-title":"Kidney diseases associated with Waldenstr\u00f6m macroglobulinemia","volume":"34","author":"Uppal","year":"2019","journal-title":"Nephrol Dial Transplant"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2015.03.035","article-title":"Kidney diseases associated with monoclonal immunoglobulin M-secreting B-cell lymphoproliferative disorders: A case series of 35 patients","volume":"66","author":"Chauvet","year":"2015","journal-title":"Am J Kidney Dis"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14279","article-title":"Renal disease related to Waldenstr\u00f6m macroglobulinaemia: Incidence, pathology and clinical outcomes","volume":"175","author":"Vos","year":"2016","journal-title":"Br J Haematol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.13041117","article-title":"Kidney involvement of patients with Waldenstr\u00f6m macroglobulinemia and other IgM-producing B cell lymphoproliferative disorders","volume":"13","author":"Higgins","year":"2018","journal-title":"Clin J Am Soc Nephrol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.18036","article-title":"Diagnosis and management of Waldenstr\u00f6m macroglobulinaemia-A British Society for Haematology guideline","volume":"197","author":"Pratt","year":"2022","journal-title":"Br J Haematol"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.6004\/jnccn.2024.0001","article-title":"Waldenstr\u00f6m macroglobulinemia\/lymphoplasmacytic lymphoma, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology","volume":"22","author":"Kumar","year":"2024","journal-title":"J Natl Compr Canc Netw"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.01600408","article-title":"Renal lesions associated with IgM-secreting monoclonal proliferations: Revisiting the disease spectrum","volume":"3","author":"Audard","year":"2008","journal-title":"Clin J Am Soc Nephrol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1080\/10428190802074619","article-title":"Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia","volume":"49","author":"Leleu","year":"2008","journal-title":"Leuk Lymphoma"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2023.03.002","article-title":"Report of Consensus Panel 6 from the 11th International Workshop on Waldenstr\u00f6m's Macroglobulinemia on management of Waldenstr\u00f6m's Macroglobulinemia related amyloidosis","volume":"60","author":"Merlini","year":"2023","journal-title":"Semin Hematol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-09-525808","article-title":"The genomic landscape of Waldenstrom macroglobulinemia is characterized by highly recurring MYD88 and WHIM-like CXCR4 mutations, and small somatic deletions associated with B-cell lymphomagenesis","volume":"123","author":"Hunter","year":"2014","journal-title":"Blood"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-09-454355","article-title":"MYD88 L265P in Waldenstr\u00f6m macroglobulinemia, immunoglobulin M monoclonal gammopathy, and other B-cell lymphoproliferative disorders using conventional and quantitative allele-specific polymerase chain reaction","volume":"121","author":"Xu","year":"2013","journal-title":"Blood"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13897","article-title":"Clonal architecture of CXCR4 WHIM-like mutations in Waldenstr\u00f6m macroglobulinaemia","volume":"172","author":"Xu","year":"2016","journal-title":"Br J Haematol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.19.02314","article-title":"Genomic landscape of Waldenstr\u00f6m macroglobulinemia and its impact on treatment strategies","volume":"38","author":"Treon","year":"2020","journal-title":"J Clin Oncol"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1002\/hon.70071","article-title":"Diagnosis and management of Waldenstrom's macroglobulinemia","volume":"43","author":"Treon","year":"2025","journal-title":"Hematol Oncol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-04-846493","article-title":"Treatment of IgM-associated immunoglobulin light-chain amyloidosis with rituximab-bendamustine","volume":"132","author":"Manwani","year":"2018","journal-title":"Blood"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-04-845396","article-title":"Treatment of AL amyloidosis with bendamustine: A study of 122 patients","volume":"132","author":"Milani","year":"2018","journal-title":"Blood"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2023.03.005","article-title":"Report of consensus panel 1 from the 11th International Workshop on Waldenstrom's Macroglobulinemia on management of symptomatic, treatment-na\u00efve patients","volume":"60","author":"Buske","year":"2023","journal-title":"Semin Hematol"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/ciy458","article-title":"Increased risk of infectious complications in older patients with indolent non-Hodgkin lymphoma exposed to bendamustine","volume":"68","author":"Fung","year":"2019","journal-title":"Clin Infect Dis"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1080\/14737140.2025.2488312","article-title":"Current and emerging treatment perspectives for adults with Waldenstr\u00f6m macroglobulinemia","volume":"25","author":"Guijosa","year":"2025","journal-title":"Expert Rev Anticancer Ther"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1002\/jha2.70019","article-title":"Bortezomib, rituximab and dexamethasone regimen (BDR) in Waldenstr\u00f6m macroglobulinaemia: A retrospective real-world analysis","volume":"6","author":"Hueso","year":"2025","journal-title":"EJHaem"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2008.20.4677","article-title":"Primary therapy of Waldenstr\u00f6m macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05-180","volume":"27","author":"Treon","year":"2009","journal-title":"J Clin Oncol"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000725","article-title":"How I treat Waldenstr\u00f6m macroglobulinemia","volume":"134","author":"Dimopoulos","year":"2019","journal-title":"Blood"},{"key":"B29","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1016\/j.hoc.2023.04.004","article-title":"Proteasome inhibitor-based regimens in the frontline management of Waldenstr\u00f6m macroglobulinemia","volume":"37","author":"Solia","year":"2023","journal-title":"Hematol Oncol Clin North Am"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1016\/j.clml.2022.02.005","article-title":"SOHO state of the art updates and next questions: Targeted therapies and emerging novel treatment approaches for Waldenstr\u00f6m macroglobulinemia","volume":"22","author":"Sermer","year":"2022","journal-title":"Clin Lymphoma Myeloma Leuk"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020006449","article-title":"Zanubrutinib for the treatment of patients with Waldenstr\u00f6m macroglobulinemia: 3 years of follow-up","volume":"136","author":"Trotman","year":"2020","journal-title":"Blood"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-09-806463","article-title":"First report of ibrutinib in IgM-related amyloidosis: Few responses, poor tolerability, and short survival","volume":"131","author":"Pika","year":"2018","journal-title":"Blood"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2018.12.003","article-title":"Autologous stem cell transplant for IgM-associated amyloid light-chain amyloidosis","volume":"25","author":"Sidiqi","year":"2019","journal-title":"Biol Blood Marrow Transplant"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-03-565135","article-title":"Treatment recommendations for patients with Waldenstr\u00f6m macroglobulinemia (WM) and related disorders: IWWM-7 consensus","volume":"124","author":"Dimopoulos","year":"2014","journal-title":"Blood"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1016\/j.clml.2020.05.021","article-title":"Efficacy of autologous and allogeneic hematopoietic cell transplantation in Waldenstr\u00f6m macroglobulinemia: A systematic review and meta-analysis","volume":"20","author":"Parrondo","year":"2020","journal-title":"Clin Lymphoma Myeloma Leuk"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705748","article-title":"Evidence of graft-versus-Waldenstrom's macroglobulinaemia effect after allogeneic stem cell transplantation: A single centre experience","volume":"40","author":"Stakiw","year":"2007","journal-title":"Bone Marrow Transplant"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1821051\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T04:29:54Z","timestamp":1781584194000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1821051\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,16]]},"references-count":36,"alternative-id":["10.3389\/fimmu.2026.1821051"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2026.1821051","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,16]]},"article-number":"1821051"}}