Roberta Fenoglio1,2, Simone Baldovino2, Michela Ferro1, Savino Sciascia1,2, Gianluca Rabajoli1, Giacomo Quattrocchio1, Giulietta Beltrame1, Carla Naretto1, Daniela Rossi1, Mirella Alpa1, Antonella Barreca3, Mario Giulio Papotti3, Dario Roccatello4,5. 1. Nephrology and Dialysis Unit and CMID, University of Turin and San Giovanni Hospital, Piazza del Donatore di Sangue 3, 10054, Turin, Italy. 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. 3. Pathology Division, Department of Oncology, University of Turin, Turin, Italy. 4. Nephrology and Dialysis Unit and CMID, University of Turin and San Giovanni Hospital, Piazza del Donatore di Sangue 3, 10054, Turin, Italy. dario.roccatello@unito.it. 5. 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. dario.roccatello@unito.it.
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.
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.
Authors: Tatiana Prokaeva; Brian Spencer; Fangui Sun; Richard M O'Hara; David C Seldin; Lawreen H Connors; Vaishali Sanchorawala Journal: Amyloid Date: 2016-09-28 Impact factor: 7.141
Authors: Eli Muchtar; Angela Dispenzieri; Martha Q Lacy; Francis K Buadi; Prashant Kapoor; Suzanne R Hayman; Wilson Gonsalves; Rahma Warsame; Taxiarchis V Kourelis; Rajshekhar Chakraborty; Stephen Russell; John A Lust; Yi Lin; Ronald S Go; Steven Zeldenrust; S Vincent Rajkumar; David Dingli; Nelson Leung; Robert A Kyle; Shaji K Kumar; Morie A Gertz Journal: Ann Med Date: 2017-03-27 Impact factor: 4.709
Authors: Shaji Kumar; Angela Dispenzieri; Martha Q Lacy; Suzanne R Hayman; Francis K Buadi; Colin Colby; Kristina Laumann; Steve R Zeldenrust; Nelson Leung; David Dingli; Philip R Greipp; John A Lust; Stephen J Russell; Robert A Kyle; S Vincent Rajkumar; Morie A Gertz Journal: J Clin Oncol Date: 2012-02-13 Impact factor: 44.544
Authors: Luis M Blancas-Mejia; Pinaki Misra; Christopher J Dick; Shawna A Cooper; Keely R Redhage; Michael R Bergman; Torri L Jordan; Khansaa Maar; Marina Ramirez-Alvarado Journal: Chem Commun (Camb) Date: 2018-09-20 Impact factor: 6.222
Authors: Jennifer H Pinney; Carol J Whelan; Aviva Petrie; Jason Dungu; Sanjay M Banypersad; Prayman Sattianayagam; Ashutosh Wechalekar; Simon D J Gibbs; Christopher P Venner; Nancy Wassef; Carolyn A McCarthy; Janet A Gilbertson; Dorota Rowczenio; Philip N Hawkins; Julian D Gillmore; Helen J Lachmann Journal: J Am Heart Assoc Date: 2013-04-22 Impact factor: 5.501