Pf625 Venetoclax for the Treatment of Translocation (11; 14) Al Amyloidosis

Abstract

The use of each treatment has noticeably changed over time (See graph). The peak treatment choices in the early 2000 s were Alkylator-or CHOP-based therapies. This has been superseded by DRC (since 2007) and R-Bendamustine (since 2011). There has been a stable role for R-mono and PA-based therapy. Regimens used at first relapse have been highly variable till 2017 when Ibrutinib became available. Treatments from 209 patients at first relapse since 1984 show that the most common category was 'Other' (52; 25%), followed by BTK Inhibitors (41; 21%), R-mono (including maintenance rituximab) (22; 11%), Alkylator-based (21; 10%), PA-based (19; 9%), DRC (19, 9%), Bendamustine-based (17, 8%), CHOP-based (13; 6%) and Bortezomib-based (5; 2%). Notably, since 2017, of the 58 patients having 2 nd line therapy 34 (58%) received BTK inhibitors. Summary/Conclusion: Observations from the Rory Morrison Registry demonstrate the evolving trends in WM treatment in the UK, including the emergence of DRC and R-Bendamustine as standard frontline therapy and a preference for BTK inhibition as 2 nd line therapy, since their availability. Further analysis is planned to assess the 'real world' impact of the emerging treatments.

Systemic amyloidosis: moving into the spotlight

“…Of 7 patients treated with venetoclax for AL amyloidosis (alone or in combinations including bortezomib and lenalidomide), 2 achieved CR and 3 achieved VGPR. However, 2 patients discontinued therapy (1 cytopenia, 1 suboptimal response) and 4 patients suffered gastrointestinal side effects 74 . A further report of 2 heavily pre-treated patients, receiving venetoclax in combination with a PI, documented a CR in both patients.…”