Improved outcomes for kidney transplantation in AL amyloidosis: impact on practice

2019 ◽  
Vol 95 (2) ◽  
pp. 258-260 ◽  
Author(s):  
Mario Nuvolone ◽  
Giampaolo Merlini
2020 ◽  
Vol 143 (4) ◽  
pp. 352-364 ◽  
Author(s):  
Foteini Theodorakakou ◽  
Despina Fotiou ◽  
Meletios A. Dimopoulos ◽  
Efstathios Kastritis

Amyloidosis comprises a diverse group of diseases characterized by misfolding of precursor proteins which eventually form amyloid aggregates and preceding intermediaries, which are deposited in target tissues causing progressive organ damage. In all forms of amyloidosis, vital organs may fail; depending on the specific amyloidosis type, this may occur rapidly or progress slowly. Beyond therapies to reduce the precursor protein (chemotherapy for light chain [AL] amyloidosis, anti-inflammatory therapy in serum A amyloid­osis [AA], and antisense RNA therapy in transthyretin amyloidosis [ATTR]), organ transplantation may also be a means to reduce amyloidogenic protein, e.g., in types of amyloid­osis in which the variant precursor is produced by the liver. Heart transplantation is a life-saving approach to the treatment of patients with advanced cardiac amyloidosis; however, amyloidosis may still be considered a contraindication to the procedure despite data supporting improved outcomes, similar to patients with other indications. Kidney transplantation is associated with particularly favorable outcomes in patients with amyloidosis, especially if the precursor protein has been eliminated. Overall, outcomes of solid organ transplantation are improving, but more data are needed to refine the selection criteria and the timing for organ transplantation, which should be performed in highly experienced centers involving multidisciplinary teams with close patient follow-up to detect amyloid recurrence.


2019 ◽  
Vol 95 (2) ◽  
pp. 405-411 ◽  
Author(s):  
Avital Angel-Korman ◽  
Lauren Stern ◽  
Shayna Sarosiek ◽  
J. Mark Sloan ◽  
Gheorghe Doros ◽  
...  

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e20568-e20568
Author(s):  
Brandon Jamaal Blue ◽  
Lauren Duncanson ◽  
Doris K. Hansen ◽  
Yumeng Zhang ◽  
Hien D Liu ◽  
...  

e20568 Background: It has been shown in mouse models doxycycline(Doxy) interferes with amyloid fibril formation. Retrospective data suggests the addition of Doxy to standard chemotherapy(chemo) reduces mortality in cardiac amyloidosis. We examined the relationship between Doxy use, treatment, and OS in a cohort of AL Amyloidosis(AL) within an academic center. Methods: AL pts between 2008 and 2020 at Moffitt Cancer Center. For this study we included patients with AL, with all types of organ involvement. We excluded pts with non-AL Amyloidosis and pts with localized amyloidosis were excluded. Data on organ involved, organ response, date of death, treatment were obtained for each pt. OS was defined as time from AL diagnosis to date of death. Univariate comparisons were performed to identify differences in treatment and other prognostic variables between Doxy groups, where appropriate (Table). Results: 209 pts that met inclusion criteria, 13 pts(6%) had cardiac involvement with AL. Of those 13 pts, only 3 pts(23%) received Doxy in addition to standard of care chemo. For this study, we included all types of AL for total 209 pts. Of the 209 pts, 9.5% received Doxy in addition to standard of care chemo and 90.5% pts did not receive Doxy and had chemo alone. Pts who received Doxy had more proteinuria (suggesting more significant renal involvement) but other characteristics were not statistically different including cardiac biomarkers (table). A numerically higher rate of organ response (67% versus 43%) was noted in patients who received Doxy but this didn’t reach statistical significance. Further the OS was numerically higher for Doxy treated pts however again this didn’t meet statistical significance. Conclusions: We noted higher organ responses and overall survival in Doxy treated pts, we could not identify statistical differences in outcomes possibly because of the sample size. Nevertheless, further follow up is warranted and Doxy may be associated with improved outcomes in AL. [Table: see text]


2019 ◽  
Vol 188 (3) ◽  
Author(s):  
Insara J. Sathick ◽  
Cara A. Rosenbaum ◽  
Victoria Gutgarts ◽  
Heather Landau

2018 ◽  
Vol 102 (2) ◽  
pp. 284-290 ◽  
Author(s):  
Blanche M. Chavers ◽  
Michelle N. Rheault ◽  
Arthur J. Matas ◽  
Scott C. Jackson ◽  
Marie E. Cook ◽  
...  

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