{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T16:37:09Z","timestamp":1791995829234,"version":"4.4.0"},"reference-count":6,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T00:00:00Z","timestamp":1727654400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T00:00:00Z","timestamp":1727654400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China,China","doi-asserted-by":"crossref","award":["81800046"],"award-info":[{"award-number":["81800046"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Nephrol"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Multiple myeloma (MM) often causes renal tubular damage, such as the light chain cast nephropathy (LCCN) and the light chain proximal tubulopathy (LCPT). The excessive light chains deposited in the proximal and distal tubules usually manifest with different characteristics, leading to a rare coexistence of the two pathological conditions. Here we report a unique case of a patient with multiple myeloma (MM) who presented with acute kidney injury (AKI) due to dual conditions of \u03bb light chain-restricted non-crystalline LCPT and LCCN. This report reviews the clinical presentation and histological findings, comparing them with previously published cases.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 49-year-old male patient was admitted with a chief complaint of \u201cfatigue, loss of appetite for 40 days and elevated blood creatinine for 10 days.\u201d In serum and urine, the \u03bb light chain level and the ratio of \u03ba to \u03bb free light chain were 1235\u00a0mg\/dl and 93.25\u00a0mg\/dl, 0.0022 and 0.0316, respectively. Additionally, serum protein electrophoresis showed an M-spike with monoclonal IgD-\u03bb. Bone marrow puncture revealed 30.5% primitive naive plasma cells, indicative of IgD-\u03bb MM. Light microscopy of kidney biopsy specimen showed periodic acid-Schiff (PAS)-negative cytoplasm in some proximal tubules and PAS-negative casts with a rigid appearance in some distal tubule lumens. On immunofluorescence, these proximal tubular epithelial cells cytoplasm and casts stained exclusively with \u03bb-light chains. Electron microscopy did not reveal any crystalline inclusions. Given the clinical and bone marrow puncture findings, the overall pathological presentation was LCPT with LCCN secondary to IgD-\u03bb MM. After chemotherapy and dialysis, the patient\u2019s condition was improved and he was tracked in follow-ups.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>In some tubular renal injuries caused by MM, the morphological changes are subtle and often overlooked. In this paper, we present a rare case of LCPT with LCCN showing \u03bb restriction in patient with MM. Through the clinicopathological analysis of patients, the understanding of the disease can be deepened and the diagnosis rate improved.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12882-024-03721-9","type":"journal-article","created":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T21:02:05Z","timestamp":1727730125000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Lambda light chain - restricted non - crystalline proximal tubulopathy with cast nephropathy in multiple myeloma: a case report and literature review"],"prefix":"10.1186","volume":"25","author":[{"given":"Mingfu","family":"Lan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaohui","family":"Guo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caiyun","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoqin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanxia","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,9,30]]},"reference":[{"issue":"5","key":"3721_CR1","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1681\/ASN.2015020185","volume":"27","author":"MB Stokes","year":"2016","unstructured":"Stokes MB, Valeri AM, Herlitz L, Khan AM, Siegel DS, Markowitz GS, D\u2019Agati VD. Light Chain Proximal Tubulopathy: clinical and pathologic characteristics in the Modern Treatment Era. J Am Soc Nephrol. 2016;27(5):1555\u201365.","journal-title":"J Am Soc Nephrol"},{"issue":"5","key":"3721_CR2","doi-asserted-by":"publisher","first-page":"1225","DOI":"10.1016\/j.ekir.2021.02.026","volume":"6","author":"C Sirac","year":"2021","unstructured":"Sirac C, Batuman V, Sanders PW. The proximal tubule toxicity of Immunoglobulin Light Chains. Kidney Int Rep. 2021;6(5):1225\u201331.","journal-title":"Kidney Int Rep"},{"issue":"6","key":"3721_CR3","doi-asserted-by":"publisher","first-page":"822","DOI":"10.1053\/j.ajkd.2019.04.029","volume":"74","author":"JJ Hogan","year":"2019","unstructured":"Hogan JJ, Alexander MP, Leung N. Dysproteinemia and the kidney: core curriculum 2019. Am J Kidney Dis. 2019;74(6):822\u201336.","journal-title":"Am J Kidney Dis"},{"issue":"7","key":"3721_CR4","doi-asserted-by":"publisher","first-page":"2049","DOI":"10.1681\/ASN.2015050581","volume":"27","author":"A Luciani","year":"2016","unstructured":"Luciani A, Sirac C, Terryn S, Javaugue V, Prange JA, Bender S, Bonaud A, Cogne M, Aucouturier P, Ronco P, et al. Impaired lysosomal function underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome.  J Am Soc Nephrol. 2016;27(7):2049\u201361.","journal-title":"J AM SOC NEPHROL"},{"issue":"7","key":"3721_CR5","doi-asserted-by":"publisher","first-page":"1810","DOI":"10.1681\/ASN.2017121319","volume":"29","author":"S Sethi","year":"2018","unstructured":"Sethi S, Rajkumar SV, D\u2019Agati VD. The complexity and heterogeneity of Monoclonal Immunoglobulin-Associated Renal diseases.  J Am Soc Nephrol. 2018;29(7):1810\u201323.","journal-title":"J AM SOC NEPHROL"},{"issue":"1","key":"3721_CR6","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1038\/ki.1995.269","volume":"48","author":"M Leboulleux","year":"1995","unstructured":"Leboulleux M, Lelongt B, Mougenot B, Touchard G, Makdassi R, Rocca A, Noel LH, Ronco PM, Aucouturier P. Protease resistance and binding of ig light chains in myeloma-associated tubulopathies. Kidney Int. 1995;48(1):72\u20139.","journal-title":"Kidney Int"}],"container-title":["BMC Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-024-03721-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12882-024-03721-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-024-03721-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T21:02:54Z","timestamp":1727730174000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcnephrol.biomedcentral.com\/articles\/10.1186\/s12882-024-03721-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,30]]},"references-count":6,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3721"],"URL":"https:\/\/doi.org\/10.1186\/s12882-024-03721-9","relation":{},"ISSN":["1471-2369"],"issn-type":[{"value":"1471-2369","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,30]]},"assertion":[{"value":"5 March 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 August 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 September 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Ethics approval and consent to participate Written informed consent was obtained. Ethical approval was obtained from the Ethics Committee of the Xijing Hospital, School of Fourth Military Medical University, China, in accordance with the ethical guidelines of the 1975 Declaration of Helsinki.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Written consent was obtained from the patient for publication of the information about him that appears within this Case Report. Informed consent was obtained from all participants.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"324"}}