Revised Mayo Clinic AL Amyloidosis Staging System
Staging system for newly-diagnosed light-chain amyloidosis, incorporating serum free light chains.
Research authors: Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Colby C, et al.
Details Formula Study characteristics Files & References
★★★★
Model author
Model ID
1925
Version
1.19
Revision date
2019-10-24
Specialty
MeSH terms
  • Amyloidoses
  • Risk Factors
  • Prognosis
  • Model type
    Linear model (Calculation)
    Status
    public
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    Formula
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    Condition Formula

    Additional information

    This prognostic staging system for newly-diagnosed AL (light chain) amyloidosis was developed from a retrospective analysis of 810 patients assessed at the Mayo Clinic.1

    On multivariate analysis (which included bone marrow plasma cell percentage, presence of circulating plasma cells, plasma cell labelling index and beta-2 microglobulin), only the serum free light chain difference, NT-proBNP and Troponin T predicted overall survival, and were used in the final staging system. Serum free light chains were determined using the Binding Site Freelite assay, and cTNT and NT-proBNP by Roche Diagnostics assays. The staging system was validated in a cohort of 303 patients who had autologous stem cell transplantation (using pre-transplantation laboratory values) and a cohort of 103 patients enrolled in clinical trials.1

    In comparison with a prior three-stage amyloidosis staging system from the Mayo Clinic, which did not incorporate serum free light chains, and which used different cTNT and NT-proBNP thresholds,2 this system offers improved discrimination.1

    References: 
    1. Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Colby C et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol. 2012; 30: 989-95.
       
    2. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004; 22: 3751-7.

    Study Population

    Total population size: 810
    Males: {{ model.numberOfMales }}
    Females: {{ model.numberOfFemales }}

    Continuous characteristics

    Name LL Q1 Median Q3 UL Unit
    Age 48 63 75 years
    Free light chains (FLC) difference 2.5 18 103 mg/dL
    Bone marrow plasma cells 4 10 30 %
    NT-proBNP 80 1800 13000 pg/mL
    Ejection fraction 40 62 72 %
    Septal thickness 10 14 18 mm
    Serum creatinine 0.8 1.1 2.2 mg/dL
    Serum albumin 1.6 2.8 3.5 g/dL

    Categorical characteristics

    Name Subset / Group Nr. of patients
    Circulating plasma cells Yes 40
    No 253

    Number of risk factors:
    ...
    risk factors

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    Result
    Note
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    Number of risk factors: risk factors

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    Outcome stratification

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    Conditional information

    Result interpretation

    This prognostic staging system for newly-diagnosed AL (light chain) amyloidosis was developed from a retrospective analysis of 810 patients assessed at the Mayo Clinic.

    Model validation: 
    The staging system was validated in a cohort of 303 patients who had autologous stem cell transplantation (using pre-transplantation laboratory values) and a cohort of 103 patients enrolled in clinical trials.1 

    Model performance: 
    In comparison with a prior three-stage amyloidosis staging system from the Mayo Clinic, which did not incorporate serum free light chains, and which used different cTNT and NT-proBNP thresholds,2 this system offers improved discrimination.1

    References: 

    1. Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Colby C et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol. 2012; 30: 989-95.
       
    2. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004; 22: 3751-7.

    {{ file.classification }}

    Calculations alone should never dictate patient care, and are no substitute for professional judgement. See our full disclaimer.

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