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Outcome of patients with severe AL amyloidosis and biopsy-proven renal involvement ineligible for bone marrow transplantation

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Abstract

Introduction

AL amyloidosis is caused by a clone of plasma cell. Due to the impact of the disease on patient survival, careful evaluation of organ involvement is essential and treatment should be tailored to single patient's risk. AIM: We analyzed the clinical, laboratory and histological characteristics of 21 elderly patients (pts) (mean age 74.7 ± 7.97 years, range 55–81) with AL amyloidosis, including 17 patients (81%) with biopsy-proven renal involvement, who were ineligible for bone marrow transplantation, and evaluated the impact of renal impairment on survival.

Results

Cardiac and renal involvement was found in 14 (67%) cases. Among the 17 patients with renal involvement, 12 had renal failure with proteinuria, and one showed isolated renal failure and vascular amyloid deposition. Hematological response occurred in 57.1% after first line therapy (75% after three cycles). In six of the patients with renal involvement, proteinuria decreased from 4.2 to 1.1 g/24 h (range 0.2–3 g/24 h), serum Creatinina (sCr) levels declined or stabilized. Severe renal failure at diagnosis was found to directly influence patient survival, while the Staging System for Renal Outcome in AL Amyloidosis did not associate with outcomes.

Conclusions

To the best of our knowledge this is the first case series in which the whole cohort of patients with urinary or functional abnormalities underwent a histological evaluation. None of the patients were eligible for bone marrow transplantation. Hematologic response was 57.1%, while renal response was much lower (35%). Of note, the Staging System did not completely apply to this peculiar setting of patients in whom renal involvement was not presumptive but biopsy-proven. More aggressive approaches may be needed in these patients to avoid the inexorable progression of the disease.

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Author notes

  1. R. Fenoglio and S. Baldovino contributed equally to this work.

Authors and Affiliations

  1. Nephrology and Dialysis Unit and CMID, University of Turin and San Giovanni Hospital, Piazza del Donatore di Sangue 3, 10054, Turin, Italy

    Roberta Fenoglio, Michela Ferro, Savino Sciascia, Gianluca Rabajoli, Giacomo Quattrocchio, Giulietta Beltrame, Carla Naretto, Daniela Rossi, Mirella Alpa & Dario Roccatello

  2. Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, University of Turin, Turin, Italy

    Roberta Fenoglio, Simone Baldovino, Savino Sciascia & Dario Roccatello

  3. Pathology Division, Department of Oncology, University of Turin, Turin, Italy

    Antonella Barreca & Mario Giulio Papotti

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  1. Roberta Fenoglio
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Correspondence to Dario Roccatello.

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Fenoglio, R., Baldovino, S., Ferro, M. et al. Outcome of patients with severe AL amyloidosis and biopsy-proven renal involvement ineligible for bone marrow transplantation. J Nephrol 34, 231–240 (2021). https://doi.org/10.1007/s40620-020-00748-7

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