Carolina R D Carmo , Madalina Uzunov , Nadia Arzouk , Frederic Charlotte , Magali Colombat , Pascal Lebray
{"title":"非淀粉样轻链沉积症的骨髓和肝肾联合移植手术:6年后病情完全缓解的独特病例报告","authors":"Carolina R D Carmo , Madalina Uzunov , Nadia Arzouk , Frederic Charlotte , Magali Colombat , Pascal Lebray","doi":"10.1016/j.liver.2024.100219","DOIUrl":null,"url":null,"abstract":"<div><p>Light Chain Deposition Disease (LCDD) is a rare monoclonal gammopathy characterized by deposition of light chains along the basement membranes of various organs, leading to progressive dysfunction (1,2). It has histological features that differ from amyloidosis and is most often characterized by the overproduction of kappa light chains (2). The kidney is usually the most affected organ and is responsible for the initial clinical manifestations, whereas liver dysfunction is less common and generally less severe than kidney involvement (1,3). We present a case of LCDD in a patient with renal dysfunction associated with secondary sclerosing cholangitis (SSC) and severe liver and kidney involvement, efficiently treated with autologous stem cell transplantation followed by double liver-kidney transplantation.</p></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"14 ","pages":"Article 100219"},"PeriodicalIF":0.0000,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666967624000205/pdfft?md5=3f38ee5f3beb9fefcfaab667b14b0f90&pid=1-s2.0-S2666967624000205-main.pdf","citationCount":"0","resultStr":"{\"title\":\"Sequential bone marrow and combined liver-kidney transplantations for a non-amyloid light chain deposition disease: A unique case report with a complete remission at 6 years\",\"authors\":\"Carolina R D Carmo , Madalina Uzunov , Nadia Arzouk , Frederic Charlotte , Magali Colombat , Pascal Lebray\",\"doi\":\"10.1016/j.liver.2024.100219\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>Light Chain Deposition Disease (LCDD) is a rare monoclonal gammopathy characterized by deposition of light chains along the basement membranes of various organs, leading to progressive dysfunction (1,2). It has histological features that differ from amyloidosis and is most often characterized by the overproduction of kappa light chains (2). The kidney is usually the most affected organ and is responsible for the initial clinical manifestations, whereas liver dysfunction is less common and generally less severe than kidney involvement (1,3). We present a case of LCDD in a patient with renal dysfunction associated with secondary sclerosing cholangitis (SSC) and severe liver and kidney involvement, efficiently treated with autologous stem cell transplantation followed by double liver-kidney transplantation.</p></div>\",\"PeriodicalId\":100799,\"journal\":{\"name\":\"Journal of Liver Transplantation\",\"volume\":\"14 \",\"pages\":\"Article 100219\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-03-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.sciencedirect.com/science/article/pii/S2666967624000205/pdfft?md5=3f38ee5f3beb9fefcfaab667b14b0f90&pid=1-s2.0-S2666967624000205-main.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Liver Transplantation\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2666967624000205\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Liver Transplantation","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2666967624000205","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Sequential bone marrow and combined liver-kidney transplantations for a non-amyloid light chain deposition disease: A unique case report with a complete remission at 6 years
Light Chain Deposition Disease (LCDD) is a rare monoclonal gammopathy characterized by deposition of light chains along the basement membranes of various organs, leading to progressive dysfunction (1,2). It has histological features that differ from amyloidosis and is most often characterized by the overproduction of kappa light chains (2). The kidney is usually the most affected organ and is responsible for the initial clinical manifestations, whereas liver dysfunction is less common and generally less severe than kidney involvement (1,3). We present a case of LCDD in a patient with renal dysfunction associated with secondary sclerosing cholangitis (SSC) and severe liver and kidney involvement, efficiently treated with autologous stem cell transplantation followed by double liver-kidney transplantation.