{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T15:35:06Z","timestamp":1791819306647,"version":"4.3.4"},"reference-count":22,"publisher":"BMJ","issue":"8","funder":[{"name":"Medical Scientific Research Project of Jiangsu Provincial Health","award":["ZD2021018"],"award-info":[{"award-number":["ZD2021018"]}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81800629"],"award-info":[{"award-number":["81800629"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82070793"],"award-info":[{"award-number":["82070793"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Clin Pathol"],"accepted":{"date-parts":[[2024,11,16]]},"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Light chain proximal tubulopathy (LCPT) is a rare complication of paraprotein-related diseases. We report a case series to present the clinicopathological characteristics and outcomes of LCPT.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A multicentre retrospective case series of 47 patients with LCPT, consisting of 36 crystalline, three non-crystalline, and eight mixed LCPTs, was studied between January 2007 and December 2023.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The median age at diagnosis was 57 years. Presentations included proteinuria (100%), renal insufficiency (62%) and Fanconi syndrome (68%). The underlying haematological diagnoses were monoclonal gammopathy of renal significance in 81% and multiple myeloma in 19%. Monoclonal light chain (LC) was detected in all cases using serum\/urine-free LC assays or immunofixation electrophoresis. Among 36 crystalline LCPTs, 34 were \u03ba-restricted and 2 \u03bb-restricted. Three non-crystalline LCPTs were all \u03bb-restricted. In mixed LCPTs, seven were \u03ba-restricted and one was \u03bb-restricted. Notably, 66% frozen-section immunofluorescence failed to reveal restricted LC, requiring paraffin-immunofluorescence or immunoelectron microscopy. The appearance of inclusions displayed intraindividual homogeneity but interindividual heterogeneity in 42 patients and notable intraindividual heterogeneity in the remaining 5 patients. Haematological complete response, very good partial response and partial response occurred in 61%. Kidney function improved or remained stable in 84%, worsened in 8% and progressed to end-stage renal disease in 8%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Proteinuria and kidney dysfunction are the most common but less-specific renal manifestations of LCPTs, with most featuring Fanconi syndrome. Crystalline LCPT, primarily associated with \u03ba-LC, is the predominant form. Most inclusions displayed intraindividual homogeneity and interindividual heterogeneity by electron microscopy. Most achieved haematological responses and favourable renal outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/jcp-2024-209620","type":"journal-article","created":{"date-parts":[[2024,12,12]],"date-time":"2024-12-12T23:06:26Z","timestamp":1734044786000},"page":"562-568","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Clinicopathological characteristics of light chain proximal tubulopathy: a multicentre case series"],"prefix":"10.1136","volume":"78","author":[{"given":"Yao","family":"Lin","sequence":"first","affiliation":[{"name":"National Clinical Research Center for Kidney Diseases, Jinling Hospital, Nanjing Medical University, Nanjing, China"},{"name":"Center for Kidney Disease, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, 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