A Changing Landscape of Mortality for Systemic Light Chain Amyloidosis

Area of Science:

• Hematology
• Cardiology
• Oncology

Background:

• Systemic light chain (AL) amyloidosis is characterized by immunoglobulin light chain deposition in organs.
• Cardiac involvement significantly increases mortality in AL amyloidosis patients.
• Historically poor survival rates may be improving due to advancements in light chain-directed therapies.

Purpose of the Study:

• To analyze mortality trends in systemic amyloidosis following the adoption of modern treatments.
• To re-evaluate the prognostic significance of components within the current staging system for AL amyloidosis.

Main Methods:

• A retrospective review of 194 patients diagnosed with AL amyloidosis between 2009 and 2016.
• Analysis of clinical data, overall survival, and the prognostic influence of staging system components.
• Utilized the most common staging system to categorize patients at diagnosis.

Main Results:

• Median overall survival for stage 3 and 4 patients was 59 and 6 months, respectively.
• Survival was not reached for stage 1 and 2 patients, indicating >50% survival by study end.
• Cardiac biomarkers (NT-proBNP, troponin I) significantly impacted prognosis, unlike serum free light chains (dFLC) ≥18 mg/dl.

Conclusions:

• Systemic AL amyloidosis patient survival has improved across all disease stages with current therapies.
• Cardiac biomarkers at diagnosis are vital prognostic indicators.
• Baseline dFLC levels ≥18 mg/dl are less significant for prognosis than previously established.