Effects of involved organs, disease stage and other parameters on prognosis, treatment response and survival in al amyloidosis patients with kidney involvement

Objective: AL amyloidosis is a systemic disease, occurring due to misfolded monoclonal immunoglobulin light chains. While cardiac involvement determines overall survival, renal involvement determines renal survival. In this study, effects of staging systems, response to treatment, presence of AF and biochemical markers on overall and renal survival were analyzed. Method: Data of 31 patients who were ≥18 years old, given ≥1 lines of therapy, followed up for ≥3 months until June 2023 at CTF Hematology Clinic were studied retrospectively. Effects of independent variables on overall and renal survival were analyzed with Kaplan-Meier. Significance p value was <0.05. Results: During follow up 29% (n=9) of patients had ESKD and 35.5% (n=11) died. Mean follow-up time until ESKD was 31.3 and mean overall follow-up time was 37.6 months. ASCT was performed at frontline therapy in 19.4% (n=6) of patients. It was proved with stratification of Mayo 2012 staging, overall survival of patients who had hematologic response was prolonged significantly. Patients with Palladini stage III had shorter renal survival than patients with Palladini stage I-II. It was shown that high levels of serum beta-2 microglobulin (>4500ng/ml), NT-proBNP (≥8500pg/ml), uric acid (>8mg/dl) and presence of AF shortened overall survival. Discussion and conclusion: Study showed effects of Palladini staging system on renal survival in renal AL amyloidosis patients. Indeed, effects of Mayo 2012 staging system, hematologic responses, presence of AF, levels of beta-2 microglobulin, uric acid and NT-proBNP on overall survival were proved. It was shown that there was a shorter overall survival in patients than healthy population.