DOAJ Open Access 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 +24 lainnya

Abstrak

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

Penulis (29)

M

Muhammad Umaid Rauf

S

Steven Law

M

Marisa Santostefano

P

Philip N. Hawkins

A

Aviva Petrie

F

Francesco Cappelli

F

Federico Perfetto

Y

Yousuf Razvi

A

Aldostefano Porcari

S

Sriram Ravichandran

A

Adam Ioannou

J

Joshua Bomsztyk

A

Alessia Argirò

C

Costanza Gaudio

E

Elisabetta Antonioli

A

Alessandro Barilaro

M

Marco Delsante

V

Vittorio Di Maso

M

Maria G. Chiappini

O

Olabisi Ogunbiyi

O

Oliver C. Cohen

A

Ana Martinez-Naharro

C

Carol Whelan

H

Helen J. Lachmann

A

Ashutosh D. Wechalekar

F

Federico Alberici

M

Marianna Fontana

M

Marco Allinovi

J

Julian D. Gillmore

Format Sitasi

Rauf, M.U., Law, S., Santostefano, M., Hawkins, P.N., Petrie, A., Cappelli, F. et al. (2025). Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis. https://doi.org/10.3324/haematol.2025.287703

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Informasi Jurnal
Tahun Terbit
2025
Sumber Database
DOAJ
DOI
10.3324/haematol.2025.287703
Akses
Open Access ✓