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Comparative Study
. 2006 Apr 15;107(8):3378-83.
doi: 10.1182/blood-2005-07-2922. Epub 2006 Jan 5.

Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation

Angela Dispenzieri  1 , Martha Q Lacy, Jerry A Katzmann, S Vincent 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, Morie A Gertz
Affiliations
Comparative Study

Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation

Angela Dispenzieri et al. Blood. .

Abstract

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 transplantation (PBSCT). Ninety-three such patients had serial FLC measurements 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 < .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 after 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 before and after PBSCT are important predictors of patient outcome.

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Figures

Figure 1.
Figure 1.
Overall survival according to baseline serum immunoglobulin free light chain level. Median value, 152 mg/L.
Figure 2.
Figure 2.
Reduction of serum immunoglobulin free light chain after peripheral blood stem cell transplantation. (A) Distribution of percent change after transplantation. (B) Overall survival by 50% reduction of immunoglobulin free light chain level from baseline. (C) Overall survival by 90% reduction of immunoglobulin free light chain level from baseline.
Figure 3.
Figure 3.
Relevance of absolute value of serum immunoglobulin free light chain after peripheral blood stem cell transplantation. (A) Distribution of levels 100 days after transplantation. (B) Overall survival by immunoglobulin free light chain at day 100. (C) Overall survival by immunoglobulin free light chain at 1 year.
Figure 4.
Figure 4.
Overall survival by FLC and hematologic complete response.
See this image and copyright information in PMC

References

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