{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T19:35:59Z","timestamp":1772998559249,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Nephrology"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Amyloidosis is a group of diseases characterised by tissue deposition of misfolded proteins, leading to organ dysfunction without appropriate therapy. Fibrinogen A \u03b1\u2010chain amyloidosis is a rare hereditary amyloidosis that can be difficult to distinguish from other causes of renal amyloidosis. We report on a 66\u2010year\u2010old female who presented with deteriorating kidney function. Serum kappa\/lambda free light chain ratio was normal with no detectable paraprotein. Kidney biopsy showed marked glomerular expansion and architectural destruction due to amyloid deposition with no vascular amyloid deposits and moderate kappa staining on immunohistochemistry. Despite bone marrow aspirate and trephine showing no evidence of plasma cell dyscrasia, light chain amyloidosis was diagnosed, and she was treated with chemotherapy, eventually progressing to kidney failure. Subsequent genetic testing revealed the amyloidogenic variant of fibrinogen A \u03b1 due to mutation Glu526Val, confirming a diagnosis of fibrinogen A \u03b1\u2010chain amyloidosis and not light chain amyloidosis. She received a deceased donor kidney transplant and was treated with doxycycline for its anti\u2010fibrillogenesis effect with no amyloid recurrence on post\u2010transplant surveillance biopsies, satisfactory kidney function and no proteinuria 6\u2009years post\u2010transplant. Renal amyloidosis cannot be attributed to the light chain subtype without evidence of plasma cell dyscrasia. Understanding of patterns of amyloid deposition in all renal amyloidosis subtypes is necessary, and genetic testing and mass spectrometry should be considered when immunohistochemistry is inconclusive. Correct subtyping of renal amyloidosis is crucial to avoid unnecessary toxicity from chemotherapy and determine the risk of recurrence for kidney transplantation. A multidisciplinary team approach with amyloidosis expertise is recommended.<\/jats:p>","DOI":"10.1111\/nep.70170","type":"journal-article","created":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T03:39:18Z","timestamp":1770089958000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Cautionary Tale: An Instructive Case of Recognising Non\u2010Amyloid Light Chain Renal Amyloidosis"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4675-1788","authenticated-orcid":false,"given":"Gayathri","family":"Bimal","sequence":"first","affiliation":[{"name":"Department of Renal Medicine Eastern Health  Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Darren","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine Eastern Health  Melbourne Victoria Australia"},{"name":"Eastern Health Clinical School Monash University  Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick","family":"Hosking","sequence":"additional","affiliation":[{"name":"Eastern Health Clinical School Monash University  Melbourne Victoria Australia"},{"name":"Department of Anatomical Pathology Eastern Health  Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon","family":"Gibbs","sequence":"additional","affiliation":[{"name":"Epworth HealthCare  Melbourne Victoria Australia"},{"name":"Alfred Health  Melbourne Victoria Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,2,2]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2023-323502"},{"key":"e_1_2_7_3_1","unstructured":"Australian Amyloidosis Network \u201cAustralasian Amyloidosis Network \u201d2025 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