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...
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Ferrata Storti Foundation
2025-06-01
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| Series: | Haematologica |
| Online Access: | https://haematologica.org/article/view/12139 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Muhammad Umaid Rauf |
| collection | DOAJ |
| description |
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 |
| format | Article |
| id | doaj-art-0129c81a967b4b64a6a8b7473cbe121c |
| institution | Matheson Library |
| issn | 0390-6078 1592-8721 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Ferrata Storti Foundation |
| record_format | Article |
| series | Haematologica |
| spelling | doaj-art-0129c81a967b4b64a6a8b7473cbe121c2025-06-26T19:49:58ZengFerrata Storti FoundationHaematologica0390-60781592-87212025-06-01999110.3324/haematol.2025.287703Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosisMuhammad Umaid Rauf0Steven Law1Marisa Santostefano2Philip N. Hawkins3Aviva Petrie4Francesco Cappelli5Federico Perfetto6Yousuf Razvi7Aldostefano Porcari8Sriram Ravichandran9Adam Ioannou10Joshua Bomsztyk11Alessia Argirò12Costanza Gaudio13Elisabetta Antonioli14Alessandro Barilaro15Marco Delsante16Vittorio Di Maso17Maria G. Chiappini18Olabisi Ogunbiyi19Oliver C. Cohen20Ana Martinez-Naharro21Carol Whelan22Helen J. Lachmann23Ashutosh D. Wechalekar24Federico Alberici25Marianna Fontana26Marco Allinovi27Julian D. Gillmore28National Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNephrology, Dialysis and Renal Transplant Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna; Alma Mater Studiorum, University of Bologna, BolognaNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonEastman Dental Institute, University College London, LondonTuscan Regional Amyloidosis Centre, Careggi University Hospital, FlorenceTuscan Regional Amyloidosis Centre, Careggi University Hospital, FlorenceNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, London, UK; Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of TriesteNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonTuscan Regional Amyloidosis Centre, Careggi University Hospital, FlorenceTuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy; Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, FlorenceTuscan Regional Amyloidosis Centre, Careggi University Hospital, FlorenceTuscan Regional Amyloidosis Centre, Careggi University Hospital, FlorenceNephrology Unit, University Hospital of Parma, ParmaDepartment of Nephrology, Cattinara Hospital, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), TriesteFatebenefratelli Foundation-'San Giovanni Calibita' Fatebenefratelli Hospital, Clinical Pathophysiology Center, RomeNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonNephrology Unit, University of Brescia, ASST Spedali Civili, BresciaNational Amyloidosis Centre, University College London, Royal Free Hospital, LondonTuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy; Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, FlorenceNational Amyloidosis Centre, University College London, Royal Free Hospital, London 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 phttps://haematologica.org/article/view/12139 |
| spellingShingle | 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 Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis Haematologica |
| title | Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis |
| title_full | Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis |
| title_fullStr | Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis |
| title_full_unstemmed | Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis |
| title_short | Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis |
| title_sort | revised renal stratification and progression models for predicting long term renal outcomes in immunoglobulin light chain amyloidosis |
| url | https://haematologica.org/article/view/12139 |
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