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    Plasma Cell precursor and Other Disorders

    Category: Plasma Cell precursor and Other Disorders

    Real-World Outcomes and Treatment Patterns in AL-Amyloidosis (AL-A) in Austria: An Analysis from the Austrian Interdiscplinary Amyloidosis Registry (AIDA)

    (PA-372) Real-World Outcomes and Treatment Patterns in Al-amyloidosis (AL-A) in Austria: An Analysis from the Austrian Interdiscplinary Amyloidosis Registry (AIDA)

    Friday, September 19, 2025
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    Introduction:      Aim : To create an overview of the treatment landscape for AL-A in Austria.

    Methods:   210 AL- pts. were recruited from Austrian Interdisciplinary Amyloidosis Registry. Statistics were descriptive.

    Results:   Pts. : 210 AL-A pts., 45.9% of pts. in AIDA, with (median follow up 7 years) were analysed. (others mostly wt-ATTR). 62.38%. of pts were male. AL-A pts. were younger (median age 66a vs. 78a) compared to wt-ATTR pts.. Organ involvement: heart (78.6%), kidneys (58.1%), GI-tract (23.3%), PNS (21.4%). Advanced Mayo stages predominant, resulting in a high number of pts. with severe to moderate renal and/or cardiac insufficiency (4.6%, 42.2% - 37.2%, 51.2% ).
    Treatments:  141/210 pts. received a 1st line of treatment (LoT), 35.5% a 2nd LoT, and 20,6% a 3rd or higher LoT, This attrition rate per LoT is above, the one for Myeloma. Median time to next treatment was 9.9 months. 54 pts. received a 1st LoT intending later ASCT, but only 54% received it. This compares inferior to Myeloma. The most frequently used therapeutic regimes were VCd-D (Andromeda) with or without Dara-maintenance resulting in a high rate of high-quality hematologic responses (CR+VGPR = 51.9%). 90 pts. received 1st LoTs not intending later ASCT. Daratumumab based regimes were the most used, but treatment outcomes were much inferior (hematologic CR+ VGPR = 18.9%). Only 40 pts. received 2nd,LoT with modest results.
    Survival:  Median survival was found 45 months and inferior in advanced stages.

    Conclusions:   

    Take home-messages:
    1. Efforts needed to improve early diagnosis
    2. Necessity to establish disease modifying therapies
    3. New therapeutics should be integrated in 1st LoTs preferentially.