Muhammad Umaid Rauf,Steven Law,Marisa Santostefano,Philip N Hawkins,Aviva Petrie,Francesco Cappelli,Federico Perfetto,Yousuf Razvi,Aldostefano Porcari,Sriram Ravichandran,Adam Ioannou,Joshua Bomsztyk,Alessia Argirò,Costanza Gaudio,Elisabetta Antonioli,Alessandro Barilaro,Marco Delsante,Vittorio Di Maso,Maria G Chiappini,Olabisi Ogunbiyi,Oliver C Cohen,Ana Martinez-Naharro,Carol Whelan,Helen J Lachmann,Ashutosh D Wechalekar,Federico Alberici,Marianna Fontana,Marco Allinovi,Julian D Gillmore
{"title":"预测免疫球蛋白轻链淀粉样变性患者长期肾脏预后的修订肾分层和进展模型。","authors":"Muhammad Umaid Rauf,Steven Law,Marisa Santostefano,Philip N Hawkins,Aviva Petrie,Francesco Cappelli,Federico Perfetto,Yousuf Razvi,Aldostefano Porcari,Sriram Ravichandran,Adam Ioannou,Joshua Bomsztyk,Alessia Argirò,Costanza Gaudio,Elisabetta Antonioli,Alessandro Barilaro,Marco Delsante,Vittorio Di Maso,Maria G Chiappini,Olabisi Ogunbiyi,Oliver C Cohen,Ana Martinez-Naharro,Carol Whelan,Helen J Lachmann,Ashutosh D Wechalekar,Federico Alberici,Marianna Fontana,Marco Allinovi,Julian D Gillmore","doi":"10.3324/haematol.2025.287703","DOIUrl":null,"url":null,"abstract":"Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing Renal Response or Renal Progression following chemotherapy after 6 months. We evaluated, in a test (N=1935) cohort of patients with renal AL amyloidosis who were followed for a median of 95 months, a modified 4-stage model where Renal Stage 2 was sub-categorized according to preserved (2A) or reduced (2B) estimated Glomerular Filtration Rate (eGFR). A hybrid model for evaluation of Renal Progression was also introduced, using an eGFR cut-off of 30ml/min/1.73 m2. These models were compared with existing models; namely those of Palladini and Kastritis, and results were validated in a multicenter cohort (N=438). The risk of progression to renal replacement therapy (RRT) increased progressively across all Renal Stages of the revised staging model (Hazard ratio [HR]: 3.25, 5.13, 10.66 for Stages 2A, 2B and 3 respectively vs Stage 1, each p.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":"70 1","pages":""},"PeriodicalIF":8.2000,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis.\",\"authors\":\"Muhammad Umaid Rauf,Steven Law,Marisa Santostefano,Philip N Hawkins,Aviva Petrie,Francesco Cappelli,Federico Perfetto,Yousuf Razvi,Aldostefano Porcari,Sriram Ravichandran,Adam Ioannou,Joshua Bomsztyk,Alessia Argirò,Costanza Gaudio,Elisabetta Antonioli,Alessandro Barilaro,Marco Delsante,Vittorio Di Maso,Maria G Chiappini,Olabisi Ogunbiyi,Oliver C Cohen,Ana Martinez-Naharro,Carol Whelan,Helen J Lachmann,Ashutosh D Wechalekar,Federico Alberici,Marianna Fontana,Marco Allinovi,Julian D Gillmore\",\"doi\":\"10.3324/haematol.2025.287703\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing Renal Response or Renal Progression following chemotherapy after 6 months. We evaluated, in a test (N=1935) cohort of patients with renal AL amyloidosis who were followed for a median of 95 months, a modified 4-stage model where Renal Stage 2 was sub-categorized according to preserved (2A) or reduced (2B) estimated Glomerular Filtration Rate (eGFR). A hybrid model for evaluation of Renal Progression was also introduced, using an eGFR cut-off of 30ml/min/1.73 m2. These models were compared with existing models; namely those of Palladini and Kastritis, and results were validated in a multicenter cohort (N=438). The risk of progression to renal replacement therapy (RRT) increased progressively across all Renal Stages of the revised staging model (Hazard ratio [HR]: 3.25, 5.13, 10.66 for Stages 2A, 2B and 3 respectively vs Stage 1, each p.\",\"PeriodicalId\":12964,\"journal\":{\"name\":\"Haematologica\",\"volume\":\"70 1\",\"pages\":\"\"},\"PeriodicalIF\":8.2000,\"publicationDate\":\"2025-06-26\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Haematologica\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.3324/haematol.2025.287703\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Haematologica","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3324/haematol.2025.287703","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis.
Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing Renal Response or Renal Progression following chemotherapy after 6 months. We evaluated, in a test (N=1935) cohort of patients with renal AL amyloidosis who were followed for a median of 95 months, a modified 4-stage model where Renal Stage 2 was sub-categorized according to preserved (2A) or reduced (2B) estimated Glomerular Filtration Rate (eGFR). A hybrid model for evaluation of Renal Progression was also introduced, using an eGFR cut-off of 30ml/min/1.73 m2. These models were compared with existing models; namely those of Palladini and Kastritis, and results were validated in a multicenter cohort (N=438). The risk of progression to renal replacement therapy (RRT) increased progressively across all Renal Stages of the revised staging model (Hazard ratio [HR]: 3.25, 5.13, 10.66 for Stages 2A, 2B and 3 respectively vs Stage 1, each p.
期刊介绍:
Haematologica is a journal that publishes articles within the broad field of hematology. It reports on novel findings in basic, clinical, and translational research.
Scope:
The scope of the journal includes reporting novel research results that:
Have a significant impact on understanding normal hematology or the development of hematological diseases.
Are likely to bring important changes to the diagnosis or treatment of hematological diseases.