Days Alive and Out of Hospital? (DAOH) in patients with AL amyloidosis according to cardiac involvement and specific treatment.

MARIA LOURDES POSADAS MARTINEZ

Simposio; XVIIIth International Symposium on Amyloidosis; 2022

Contribution ID : 117Track / Type : ISA 2022 Abstract SubmissionFormat : Undecided (Oral or Poster Presentation)Title : Morbidity and mortality measured through ?Days Alive and Out of Hospital? (DAOH) in patients with AL amyloidosis according to cardiac involvement and specific treatment.Author(s) : Posadas Martinez, María Lourdes; Vaena, Mariana; Epstein, Teo; Osorno, María Sol; Brulc, Erika Bárbara; Carretero, Marcelina; Sáez, María Soledad; Sorroche, Patricia Beatriz; Pollán, Javier; Aguirre, María Adela; Nucifora, Elsa MercedesBackground: cardiac amyloidosis is the main cause of morbidity and mortality in patients with systemic amyloidosis. Days alive and out of hospital (DAOH) is a novel patient-centered outcome that assesses the burden of disease, through the measurement of morbidity and mortality.Objectives: To describe DAOH in patients with AL amyloidosis by cardiac compromise and autologous stem cell transplantation (ASCT).Materials & Methods: Prospective cohort of consecutive patients diagnosed as AL from the Institutional Registry of Amyloidosis (RIA) from the Health Maintenance Organizations (HMO) of the Italian Hospital of Buenos Aires between 01/01/2010 and 31/08/2021. Patients with follow-up in another institution or less than 365 days were excluded. Quantitative variables were described with their median and interquartile range, and categorical variables with absolute and relative frequencies. We calculated DAOH at 1, 3, and 5 years, subtracting days hospitalized and days dead from the potential follow-up time. Only patients with a follow-up equal to or greater than the potential follow-up time were included in each group. We use Kruskal Wallis to compare DAOH between cardiac and non-cardiac compromise and ASCT and non-ASCT.Results & Discussion: 78 patients were eligible, of whom 48 were included. Of these, the total completed the follow-up for DAOH at 1 year, 32 for DAOH at 3 years and 22 for DAOH at 5 years (Figure 1). The median age was 64 years, 54% were women and the median Charlson score was 5 (IR 3-7). The most frequently affected organs were the kidney (60%) and heart (56%). Eighty-seven percent of patients received specific pharmacological treatment, 35% received ASCT and 15% heart transplantation. Ninety-two percent had at least one episode of hospitalization during the follow-up time. The median number of hospitalizations was 3 (IR 1-4). The median DAOH was 346 (IR 274-365), 1042 (IR 231-1083), and 1739 days (IR 352-1793) at 1, 3, and 5 years, respectively. At 1 year, the median of DAOH was 347 (IR 333-363) and 362 days (IR 313-365) in those with and without ASCT, respectively (p 0.33). At 5 years, the median of DAOH was 1787 (IR 1759-1804) and 1558 days (IR 595-1793) in those with and without ASCT, respectively (p 0.04). Mortality was 12% vs 56% in those with and without ASCT, respectively. At 5 years, the median of DAOH was 1042 (IR 431-1783) and 1768 days (IR 1627-1820) in those with and without cardiac involvement, respectively (p 0.04).In the present study we found that DAOH differs significantly between patients with cardiac involvement and those who received ASCT.Regarding cardiac involvement, our results coincide with the multiple studies that evaluate cardiac involvement as a key prognostic factor in traditional overall survival studies [1,2].We consider that the ASCT is an excellent evolutionary parameter regarding the patient's journey. Despite requiring a longer hospital stay during and after, reducing DAOH at 1 year, it has a long-term benefit, demonstrated by greater DAOH at 5 years in patients who received ASCT.Summary & Conclusion: Days alive and out of hospital is a measure of patient-centered outcomes that describes the patient's disease journey. This is the first paper that describes DAOH in amyloidosis AL, but this outcome may become a relevant measure for chronic disease follow-up.Keywords: Inmunoglobulin light chains; cardiac amyloidosis