Haematologica (Jun 2025)
Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis
- 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
Affiliations
- Muhammad Umaid Rauf
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Steven Law
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Marisa Santostefano
- Nephrology, Dialysis and Renal Transplant Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna; Alma Mater Studiorum, University of Bologna, Bologna
- Philip N. Hawkins
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Aviva Petrie
- Eastman Dental Institute, University College London, London
- Francesco Cappelli
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence
- Federico Perfetto
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence
- Yousuf Razvi
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Aldostefano Porcari
- National 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 Trieste
- Sriram Ravichandran
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Adam Ioannou
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Joshua Bomsztyk
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Alessia Argirò
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence
- Costanza Gaudio
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy; Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, Florence
- Elisabetta Antonioli
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence
- Alessandro Barilaro
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence
- Marco Delsante
- Nephrology Unit, University Hospital of Parma, Parma
- Vittorio Di Maso
- Department of Nephrology, Cattinara Hospital, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), Trieste
- Maria G. Chiappini
- Fatebenefratelli Foundation-'San Giovanni Calibita' Fatebenefratelli Hospital, Clinical Pathophysiology Center, Rome
- Olabisi Ogunbiyi
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Oliver C. Cohen
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Ana Martinez-Naharro
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Carol Whelan
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Helen J. Lachmann
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Ashutosh D. Wechalekar
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Federico Alberici
- Nephrology Unit, University of Brescia, ASST Spedali Civili, Brescia
- Marianna Fontana
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- Marco Allinovi
- Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy; Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, Florence
- Julian D. Gillmore
- National Amyloidosis Centre, University College London, Royal Free Hospital, London
- DOI
- https://doi.org/10.3324/haematol.2025.287703
- Journal volume & issue
-
Vol. 999,
no. 1
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
