{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T16:08:40Z","timestamp":1790784520376,"version":"4.1.0"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Mie University","id":[{"id":"https:\/\/ror.org\/01529vy56","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["CEN Case Rep"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>We report a 55-year-old man who developed nephrotic syndrome 52 months after allogeneic hematopoietic stem cell transplantation for acute lymphoblastic leukemia. He had chronic graft-versus-host disease controlled with low-dose prednisolone and tacrolimus. Asymptomatic proteinuria was detected five months before referral, followed by progressive edema, massive pleural effusion, and ascites. At presentation, laboratory testing showed profound hypoalbuminemia, mildly reduced kidney function, and nephrotic-range proteinuria. Kidney biopsy demonstrated membranous nephropathy, with periodic acid-methenamine silver staining showing spikes and bubbling of the glomerular basement membrane. Immunofluorescence showed granular capillary-wall immunoglobulin G staining with immunoglobulin G1 predominance over immunoglobulin G4, and protocadherin FAT1 immunofluorescence showed granular staining along the glomerular basement membrane. Prednisolone was increased from 5 to 30 milligrams per day while tacrolimus was continued, resulting in improved proteinuria, serum albumin, edema, and kidney function at five months without clinically significant infectious complications. We also reviewed 29 reported cases of protocadherin FAT1-associated membranous nephropathy after hematopoietic stem cell transplantation, including the present case. The review showed variable onset timing, substantial proteinuria at presentation, inconsistent immunoglobulin G4 positivity, and heterogeneous treatment with corticosteroids, rituximab, calcineurin inhibitors, and other regimens. Our case and review indicate that this disease can present late, cannot be excluded by non-immunoglobulin G4-predominant staining, and remains heterogeneous in diagnostic evaluation, treatment, and outcomes. Further accumulation of cases is needed to clarify the clinical spectrum and optimal management of this disease.<\/jats:p>","DOI":"10.1007\/s13730-026-01188-1","type":"journal-article","created":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T15:36:39Z","timestamp":1790782599000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["FAT1-associated membranous nephropathy after hematopoietic stem cell transplantation: a case report and review of the literature"],"prefix":"10.1007","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-4132-6648","authenticated-orcid":false,"given":"Ryosuke","family":"Saiki","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kan","family":"Katayama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuto","family":"Matsui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiro","family":"Murata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaoru","family":"Dohi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,30]]},"reference":[{"key":"1188_CR1","doi-asserted-by":"publisher","first-page":"975","DOI":"10.1016\/j.bbmt.2015.12.014","volume":"22","author":"O Beyar-Katz","year":"2016","unstructured":"Beyar-Katz O, Davila EK, Zuckerman T, Fineman R, Haddad N, Okasha D, Henig I, Leiba R, Rowe JM, Ofran Y. Adult nephrotic syndrome after hematopoietic stem cell transplantation: renal pathology is the best predictor of response to therapy. Biol Blood Marrow Transpl. 2016;22:975\u201381.","journal-title":"Biol Blood Marrow Transpl"},{"key":"1188_CR2","doi-asserted-by":"publisher","first-page":"1033","DOI":"10.1681\/ASN.2021111488","volume":"33","author":"S Sethi","year":"2022","unstructured":"Sethi S, Madden B, Casal Moura M, Nasr SH, Klomjit N, Gross L, Negron V, Charlesworth MC, Alexander MP, Leung N, Specks U, Fervenza FC, Haas M. Hematopoietic stem cell transplant\u2013membranous nephropathy is associated with protocadherin FAT1. J Am Soc Nephrol. 2022;33:1033\u201344.","journal-title":"J Am Soc Nephrol"},{"key":"1188_CR3","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1681\/ASN.0000000881","volume":"37","author":"AO Oteyola","year":"2026","unstructured":"Oteyola AO, Chan B, Quintar AA, Riascos-Bernal DF, Ross MJ, Sibinga NES, Chen W. Emerging role of FAT atypical cadherin 1 in kidney function and disease. J Am Soc Nephrol. 2026;37:186\u20139.","journal-title":"J Am Soc Nephrol"},{"key":"1188_CR4","doi-asserted-by":"publisher","first-page":"1003","DOI":"10.1046\/j.1523-1755.2001.0590031003.x","volume":"59","author":"T Inoue","year":"2001","unstructured":"Inoue T, Yaoita E, Kurihara H, Shimizu F, Sakai T, Kobayashi T, Ohshiro K, Kawachi H, Okada H, Suzuki H, Kihara I, Yamamoto T. FAT is a component of glomerular slit diaphragms. Kidney Int. 2001;59:1003\u201312.","journal-title":"Kidney Int"},{"key":"1188_CR5","doi-asserted-by":"publisher","first-page":"sfag005","DOI":"10.1093\/ckj\/sfag005","volume":"19","author":"MC Nackenhorst","year":"2026","unstructured":"Nackenhorst MC, Milovanovic D, Schmidt A, Mongera N, K\u00f6ller M, Passler W, Cejka D, Kozakowski N. Protocadherin FAT1-associated membranous nephropathy after hematopoietic stem cell transplantation\u2013a cohort study with clinico-pathological correlations. Clin Kidney J. 2026;19:sfag005.","journal-title":"Clin Kidney J"},{"key":"1188_CR6","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1186\/s12882-024-03675-y","volume":"25","author":"I Bosni\u0107 Kova\u010di\u0107","year":"2024","unstructured":"Bosni\u0107 Kova\u010di\u0107 I, Mato\u0161evi\u0107 M, Laganovi\u0107 M, Dika \u017d, Fi\u0161trek Prli\u0107 M, Ivandi\u0107 E, \u0106ori\u0107 M, Bulimba\u0161i\u0107 S, Durakovi\u0107 N, Peri\u0107 Z, Desnica L, Vrhovac R, Jelakovi\u0107 B, Sethi S. Vukovi\u0107 Brinar I. Specific antigen-based stratification of membranous nephropathy in patients after haematopoietic stem cell allotransplantation - a case series and literature review. BMC Nephrol. 2024;25:254.","journal-title":"BMC Nephrol"},{"key":"1188_CR7","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1681\/ASN.2016070776","volume":"28","author":"AS De Vriese","year":"2017","unstructured":"De Vriese AS, Glassock RJ, Nath KA, Sethi S, Fervenza FC. A proposal for a serology-based approach to membranous nephropathy. J Am Soc Nephrol. 2017;28:421\u201330.","journal-title":"J Am Soc Nephrol"},{"key":"1188_CR8","doi-asserted-by":"publisher","first-page":"1153","DOI":"10.1007\/s40620-023-01786-7","volume":"37","author":"N Mongera","year":"2024","unstructured":"Mongera N, Passler W, Sethi S, Kozakowski N, Tabb\u00ec MG. A case of protocadherin FAT1-positive membranous nephropathy secondary to hematopoietic stem-cell transplantation. J Nephrol. 2024;37:1153\u20136.","journal-title":"J Nephrol"},{"key":"1188_CR9","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1038\/s41467-023-36068-0","volume":"14","author":"L Seifert","year":"2023","unstructured":"Seifert L, Zahner G, Meyer-Schwesinger C, Hickstein N, Dehde S, Wulf S, K\u00f6llner SMS, Lucas R, Kylies D, Froembling S, Zielinski S, Kretz O, Borodovsky A, Biniaminov S, Wang Y, Cheng H, Koch-Nolte F, Zipfel PF, Hopfer H, Puelles VG, Panzer U, Huber TB, Wiech T, Tomas NM. The classical pathway triggers pathogenic complement activation in membranous nephropathy. Nat Commun. 2023;14:473.","journal-title":"Nat Commun"},{"key":"1188_CR10","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.cca.2018.06.050","volume":"485","author":"MF Zhang","year":"2018","unstructured":"Zhang MF, Cui Z, Zhang YM, Qu Z, Wang X, Wang F, Meng LQ, Cheng XY, Liu G, Zhao MH. Clinical and prognostic significance of glomerular C1q deposits in primary MN. Clin Chim Acta. 2018;485:152\u20137.","journal-title":"Clin Chim Acta"},{"key":"1188_CR11","doi-asserted-by":"publisher","first-page":"2764","DOI":"10.1182\/blood.V74.8.2764.2764","volume":"74","author":"AJ Mitus","year":"1989","unstructured":"Mitus AJ, Stein R, Rappeport JM, Antin JH, Weinstein HJ, Alper CA, Smith BR. Monoclonal and oligoclonal gammopathy after bone marrow transplantation. 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A copy of the written consent form is available for review by the editor of the journal.","order":3,"name":"Ethics","label":"Consent for publication","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":4,"name":"Ethics","label":"Human and animal rights","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"144"}}