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Updated: Sep 24, 2025

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
A UK consensus algorithm for early treatment modification in newly diagnosed systemic light-chain amyloidosis
Sriram Ravichandran1, Shameem Mahmood1,2, Brenden Wisniowski1,2
1National Amyloidosis Centre, London, UK.
Patients with high difference in involved and uninvolved serum free light chains (dFLC) and no response at 1 month in AL amyloidosis are unlikely to improve. Early treatment changes are suggested for these patients.
Area of Science:
- Hematology
- Oncology
- Nephrology
Background:
- Prognosis in amyloid light-chain (AL) amyloidosis is determined by depth of response.
- Identifying patients unlikely to achieve response is crucial for timely therapeutic adjustment.
Purpose of the Study:
- To identify baseline characteristics and early response markers predicting poor treatment outcome in AL amyloidosis.
- To guide early treatment modification for patients with limited response.
Main Methods:
- Multivariate analysis of baseline characteristics and 1-month response in AL amyloidosis patients.
- Assessment of difference in involved and uninvolved serum free light chains (dFLC) as a predictive marker.
Main Results:
- Elevated dFLC (>400 mg/l) at diagnosis and lack of response at 1 month significantly predicted no improvement (OR 4.051 and 4.787, respectively; p<0.005).
- Only 5% of patients with both high dFLC and no 1-month response showed subsequent improvement (p<0.005).
Conclusions:
- High dFLC at diagnosis and absence of response at 1 month are strong predictors of poor prognosis in AL amyloidosis.
- These findings support early treatment modification in non-responding patients, considering their functional status.
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