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Blood, 15 April 2006, Vol. 107, No. 8, pp. 3378-3383.
Prepublished online as a Blood First Edition Paper on January 5, 2006; DOI 10.1182/blood-2005-07-2922.
 Previous Article | Next Article 
Submitted July 21, 2005
Accepted November 7, 2005
Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplant
Angela Dispenzieri*, Martha Q Lacy, Jerry A Katzmann, S V Rajkumar, Roshini S Abraham, Suzanne R Hayman, Shaji K Kumar, Raynell Clark, Robert A Kyle, Mark R Litzow, David J Inwards, Stephen M Ansell, Ivana M Micallef, Luis F Porrata, Michelle A Elliott, Patrick B Johnston, Philip R Greipp, Thomas E Witzig, Steven R Zeldenrust, Stephen J Russell, Dennis Gastineau, and Morie A Gertz
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN, USA; Division of Clinical Biochemistry and Immunology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN, USA
Division of Clinical Biochemistry and Immunology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN, USA; Molecular Medicine, Mayo Clinic, Rochester, MN, USA
* Corresponding author; email: dispenzieri.angela{at}mayo.edu.
The immunoglobulin free light chain (FLC) is the precursor protein of amyloid in primary systemic amyloidosis (AL). Historically, the ability to monitor the amyloid protein precursor protein has been crude. We evaluated the utility of the FLC assay in a retrospective analysis of patients with AL undergoing peripheral blood stem cell transplant (PBSCT). Ninety-three such patients had serial FLC measurements (FreeliteTM) performed. The prognostic effects of the initial concentration and the extent of reduction of monoclonal FLC on survival were studied. There was a significantly higher risk of death in patients with higher baseline FLC (hazard ratio 2.6, p< 0.04). Baseline FLC correlated with serum cardiac troponin levels, and higher FLC levels were associated with more organs involved by amyloid, suggesting that high FLC levels may be associated with more advanced disease. The percent FLC reduction did not predict for survival, but the absolute level of FLC achieved after therapy did. Normalization of FLC level post-PBSCT predicted for both organ response and complete hematologic response. Achievement of FLC response was a better predictor of survival than achievement of complete hematologic response or normalization of the FLC ratio. FLC measurements both pre- and post-PBSCT are important predictors of patient outcome.
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