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

Angela Dispenzieri, Martha 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 Maxted Ansell, Ivana Micallef, Luis F. Porrata, Michelle A. Elliott, Patrick Bruce Johnston, Philip R. Greipp, Thomas Elmer Witzig, Steven R. Zeldenrust, Stephen J Russell & 2 others Dennis Gastineau, Morie Gertz

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)3378-3383
Number of pages6
JournalBlood
Volume107
Issue number8
DOIs
StatePublished - Apr 15 2006

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Immunoglobulin Light Chains
Peripheral Blood Stem Cell Transplantation
Stem cells
Immunoglobulins
Blood
Light
Amyloid beta-Protein Precursor
Primary amyloidosis
Survival
Troponin
Aptitude
Protein Precursors
Amyloidosis
Amyloid

ASJC Scopus subject areas

  • Hematology

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Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. / Dispenzieri, Angela; Lacy, Martha; Katzmann, Jerry A.; Rajkumar, S Vincent; Abraham, Roshini S.; Hayman, Suzanne R.; Kumar, Shaji K; Clark, Raynell; Kyle, Robert A.; Litzow, Mark R; Inwards, David J.; Ansell, Stephen Maxted; Micallef, Ivana; Porrata, Luis F.; Elliott, Michelle A.; Johnston, Patrick Bruce; Greipp, Philip R.; Witzig, Thomas Elmer; Zeldenrust, Steven R.; Russell, Stephen J; Gastineau, Dennis; Gertz, Morie.

In: Blood, Vol. 107, No. 8, 15.04.2006, p. 3378-3383.

Research output: Contribution to journalArticle

Dispenzieri, Angela ; Lacy, Martha ; Katzmann, Jerry A. ; Rajkumar, S Vincent ; Abraham, Roshini S. ; Hayman, Suzanne R. ; Kumar, Shaji K ; Clark, Raynell ; Kyle, Robert A. ; Litzow, Mark R ; Inwards, David J. ; Ansell, Stephen Maxted ; Micallef, Ivana ; Porrata, Luis F. ; Elliott, Michelle A. ; Johnston, Patrick Bruce ; Greipp, Philip R. ; Witzig, Thomas Elmer ; Zeldenrust, Steven R. ; Russell, Stephen J ; Gastineau, Dennis ; Gertz, Morie. / Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. In: Blood. 2006 ; Vol. 107, No. 8. pp. 3378-3383.
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AU - Gertz, Morie

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N2 - 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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