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Serum Cardiac Troponins and N-Terminal Pro-Brain Natriuretic Peptide: A Staging System for Primary Systemic Amyloidosis

From the Division of Hematology and Internal Medicine, the Division of Biostatistics, the Division of Cardiovascular Diseases and Internal Medicine, the Division of Laboratory Genetics and Laboratory Medicine and Pathology, the Department of Surgery, and the Division of Clinical Biochemistry and Immunology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN

Address reprint requests to Angela Dispenzieri, MD, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: dispenzieri.angela{at}mayo.edu

PURPOSE: Primary systemic amyloidosis (AL) is a multisystemic disorder resulting from an underlying plasma cell dyscrasia. There is no formal staging system for AL, making comparisons between studies and treatment centers difficult. Our group previously identified elevated serum cardiac troponin T (cTnT) as the most powerful predictor of overall survival. Others have reported that N-terminal pro-brain natriuretic peptide (NT-proBNP) is a valuable prognostic marker. We sought to develop a staging system for patients with AL.

PATIENTS AND METHODS: Two hundred forty-two patients with newly diagnosed AL who were seen at the Mayo Clinic between April 1979 and November 2000, and who had echocardiograms and stored serum samples at presentation were eligible for this retrospective review. NT-proBNP measurements were performed on 242 patients in whom cTnT and cardiac troponin I (cTnI) had been previously run. Two prognostic models were designed using threshold values of NT-proBNP and either cTnT or cTnI (NT-proBNP < 332 ng/L, cTnT < 0.035 µg/L, and cTnI < 0.1 µg/L). Depending on whether NT-proBNP and troponin levels were both low, were high for only one level, or were both high, patients were classified as stage I, II, or III, respectively.

RESULTS: Using the cTnT+NT-proBNP model 33%, 30%, and 37% of patients were stages I, II, and III, respectively, with median survivals of 26.4, 10.5, and 3.5 months, respectively. The alternate cTnI+NT-proBNP model predicted median survivals of 27.2, 11.1, and 4.1 months, respectively.

CONCLUSION: Stratification of AL patients into three stages is possible with two readily available and reproducible tests setting the stage for more consistent and reliable cross comparisons of therapeutic outcomes.

Supported in part by grant Nos. CA 62242 (R.A.K) and CA 91561 (A.D.) from the National Cancer Institute and the Robert A. Kyle Hematology Malignancies Fund, Mayo Foundation.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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