Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements Full Text
Journal of Clinical Oncology, 02/27/2012
Kumar S et al. – Incorporation of serum FLC-diff into the current staging system improves risk stratification for patients with AL amyloidosis and will help develop risk-adapted therapies for AL amyloidosis.
Methods- Developed a prognostic model based on 810 patients with newly diagnosed AL amyloidosis, which was further examined in 2 other datasets: 303 patients undergoing stem-cell transplantation, and 103 patients enrolled onto different clinical trials
- Examined prognostic value of plasma cell–related characteristics (ie, difference between involved and uninvolved light chain [FLC-diff], marrow plasma cell percentage, circulating plasma cells, plasma cell labeling index, and β2 microglobulin)
- Multivariate model that included these characteristics as well as cTnT and NT-ProBNP, only FLC-diff, cTnT, and NT-ProBNP were independently prognostic for OS
- Patients were assigned a score of 1 for each of FLC-diff ≥ 18 mg/dL, cTnT ≥ 0.025 ng/mL, and NT-ProBNP ≥ 1,800 pg/mL, creating stages I to IV with scores of 0 to 3 points
- Proportions of patients with stages I, II, III and IV disease 189 (25%), 206 (27%), 186 (25%) and 177 (23%), and their median OS from diagnosis was 94.1, 40.3, 14, and 5.8 months
