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    Plasma Cell precursor and Other Disorders

    Category: Plasma Cell precursor and Other Disorders

    CLINICAL PROFILE, TREATMENT PATTERNS, OUTCOMES AND PREDICTORS OF SURVIVAL IN AL AMYLOIDOSIS: A RETROSPECTIVE STUDY FROM A TERTIARY CANCER CENTER

    (PA-365) Clinical Profile, Treatment Patterns, Outcomes and Predictors of Survival in Al Amyloidosis: A Retrospective Study from a Tertiary Cancer Center

    Wednesday, September 17, 2025

    Introduction:      Light Chain (AL) amyloidosis is the most common form of systemic amyloidosis, characterized by the deposition of misfolded monoclonal light chains or their fragments in tissues, leading to progressive organ dysfunction. The clinical presentation is heterogeneous, and data from low- and middle-income countries (LMICs) remain scarce, particularly in settings with limited access to novel agents and autologous stem cell transplantation (ASCT).

    Methods:   

    We conducted a retrospective observational study of patients diagnosed with AL amyloidosis at our institution between January 2013 and April 2025. Demographic details, clinical features, treatment regimens, and outcomes were extracted from electronic medical records and analysed.

    Results:   

    A total of 93 patients were included. The median age at diagnosis was 60 years (range: 29–85), with 68.8% (n = 64) being male. Renal involvement was the most common (71%), followed by cardiac involvement (63.4%). The median duration of symptoms prior to diagnosis was 6 months (range: 1–24 months). Of the 61 patients evaluable for staging, 30.6% (n = 19) were classified as Revised Mayo Stage III or IV. VCD-based induction therapy was administered to 53.8% (n = 50) of patients; only 2.1% (n = 2) received Daratumumab-VCD, and ASCT was performed in 6.4% (n = 6). Haematological responses post-induction was observed in 89.6%, with complete response (CR) in 24.1%, very good partial response (VGPR) in 41.4%, and partial response (PR) in 24.1%. At a median follow-up of 18 months (range: 4–145), the 24-month progression-free survival (PFS) was 58% (95% CI: 45%–71%), while the 24-month and 60-month overall survival (OS) rates were 72% (95% CI: 62%–82%) and 59% (95% CI: 48%–70%), respectively. Early mortality (within 6 months of diagnosis) occurred in 41.6% (n = 10) of the total deaths. On univariate and multivariate analyses, renal involvement (HR: 3.85; 95% CI: 1.14–12.93; P = 0.029) and lack of haematological response post-induction (HR: 4.2; 95% CI: 1.2–14.92; P = 0.022) were significantly associated with inferior outcomes.

    Conclusions:   This study provides key insights into the presentation and outcomes of AL amyloidosis in an Indian cohort, highlighting delayed diagnosis, frequent renal and cardiac involvement, and limited access to advanced therapies including ASCT and daratumumab. High rates of early mortality underscore the need for timely diagnosis, aggressive supportive care, and access to novel agents. Renal involvement and failure to achieve hematologic response emerged as significant predictors of poor prognosis.