Serum immunoglobulin free light-chain measurement in primary amyloidosis: Prognostic value and correlations with clinical features

Shaji Kumar; Angela Dispenzieri; Jerry A. Katzmann; Dirk R. Larson; Colin L. Colby; Martha Q. Lacy; Suzanne R. Hayman; Francis K. Buadi; Nelson Leung; Steve R. Zeldenrust; Marina Ramirez-Alvarado; Raynell J. Clark; Robert A. Kyle; S. Vincent Rajkumar; Morie A. Gertz

doi:10.1182/blood-2010-06-290668

Serum immunoglobulin free light-chain measurement in primary amyloidosis: Prognostic value and correlations with clinical features

Shaji Kumar, Angela Dispenzieri, Jerry A. Katzmann, Dirk R. Larson, Colin L. Colby, Martha Q. Lacy, Suzanne R. Hayman, Francis K. Buadi, Nelson Leung, Steve R. Zeldenrust, Marina Ramirez-Alvarado, Raynell J. Clark, Robert A. Kyle, S. Vincent Rajkumar, Morie A. Gertz

Research output: Contribution to journal › Article › peer-review

112 Scopus citations

Abstract

Immunoglobulin free light chains (FLCs) are the precursors of amyloid fibrils in primary amyloidosis (AL). We studied the relationship between FLC levels and clinical features in 730 patients with newly diagnosed AL. The plasma cell clone was λ in 72% patients, and κ in 28% patients. κ-AL had more GI tract and liver involvement, where as renal involvement was more with λ-AL. While the overall survival (OS) was similar for κ and λ-AL, the median OS for those without an identifiable serum heavy chain was significantly shorter (12.6 vs 29.9 months; P = .02). The OS was shorter among those with a higher dFLC (involved FLC-uninvolved FLC; κ > 29.4 mg/dL or λ > 18.2 mg/dL using median for cutoff); 10.9 vs 37.1 months; P < .001. In multivariate analysis, dFLC was independent of other prognostic factors. The type of light chain impacts the spectrum of organ involvement and the FLC burden correlates with survival in AL.

Original language	English (US)
Pages (from-to)	5126-5129
Number of pages	4
Journal	Blood
Volume	116
Issue number	24
DOIs	https://doi.org/10.1182/blood-2010-06-290668
State	Published - Dec 9 2010

ASJC Scopus subject areas

Biochemistry
Immunology
Hematology
Cell Biology

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Kumar, S., Dispenzieri, A., Katzmann, J. A., Larson, D. R., Colby, C. L., Lacy, M. Q., Hayman, S. R., Buadi, F. K., Leung, N., Zeldenrust, S. R., Ramirez-Alvarado, M., Clark, R. J., Kyle, R. A., Rajkumar, S. V., & Gertz, M. A. (2010). Serum immunoglobulin free light-chain measurement in primary amyloidosis: Prognostic value and correlations with clinical features. Blood, 116(24), 5126-5129. https://doi.org/10.1182/blood-2010-06-290668

Kumar, S , Dispenzieri, A, Katzmann, JA, Larson, DR, Colby, CL, Lacy, MQ, Hayman, SR, Buadi, FK, Leung, N, Zeldenrust, SR, Ramirez-Alvarado, M, Clark, RJ, Kyle, RA, Rajkumar, SV & Gertz, MA 2010, 'Serum immunoglobulin free light-chain measurement in primary amyloidosis: Prognostic value and correlations with clinical features', Blood, vol. 116, no. 24, pp. 5126-5129. https://doi.org/10.1182/blood-2010-06-290668

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title = "Serum immunoglobulin free light-chain measurement in primary amyloidosis: Prognostic value and correlations with clinical features",

abstract = "Immunoglobulin free light chains (FLCs) are the precursors of amyloid fibrils in primary amyloidosis (AL). We studied the relationship between FLC levels and clinical features in 730 patients with newly diagnosed AL. The plasma cell clone was λ in 72% patients, and κ in 28% patients. κ-AL had more GI tract and liver involvement, where as renal involvement was more with λ-AL. While the overall survival (OS) was similar for κ and λ-AL, the median OS for those without an identifiable serum heavy chain was significantly shorter (12.6 vs 29.9 months; P = .02). The OS was shorter among those with a higher dFLC (involved FLC-uninvolved FLC; κ > 29.4 mg/dL or λ > 18.2 mg/dL using median for cutoff); 10.9 vs 37.1 months; P < .001. In multivariate analysis, dFLC was independent of other prognostic factors. The type of light chain impacts the spectrum of organ involvement and the FLC burden correlates with survival in AL.",

author = "Shaji Kumar and Angela Dispenzieri and Katzmann, {Jerry A.} and Larson, {Dirk R.} and Colby, {Colin L.} and Lacy, {Martha Q.} and Hayman, {Suzanne R.} and Buadi, {Francis K.} and Nelson Leung and Zeldenrust, {Steve R.} and Marina Ramirez-Alvarado and Clark, {Raynell J.} and Kyle, {Robert A.} and Rajkumar, {S. Vincent} and Gertz, {Morie A.}",

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AU - Colby, Colin L.

AU - Lacy, Martha Q.

AU - Hayman, Suzanne R.

AU - Buadi, Francis K.

AU - Leung, Nelson

AU - Zeldenrust, Steve R.

AU - Ramirez-Alvarado, Marina

AU - Clark, Raynell J.

AU - Kyle, Robert A.

AU - Rajkumar, S. Vincent

AU - Gertz, Morie A.

PY - 2010/12/9

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N2 - Immunoglobulin free light chains (FLCs) are the precursors of amyloid fibrils in primary amyloidosis (AL). We studied the relationship between FLC levels and clinical features in 730 patients with newly diagnosed AL. The plasma cell clone was λ in 72% patients, and κ in 28% patients. κ-AL had more GI tract and liver involvement, where as renal involvement was more with λ-AL. While the overall survival (OS) was similar for κ and λ-AL, the median OS for those without an identifiable serum heavy chain was significantly shorter (12.6 vs 29.9 months; P = .02). The OS was shorter among those with a higher dFLC (involved FLC-uninvolved FLC; κ > 29.4 mg/dL or λ > 18.2 mg/dL using median for cutoff); 10.9 vs 37.1 months; P < .001. In multivariate analysis, dFLC was independent of other prognostic factors. The type of light chain impacts the spectrum of organ involvement and the FLC burden correlates with survival in AL.

AB - Immunoglobulin free light chains (FLCs) are the precursors of amyloid fibrils in primary amyloidosis (AL). We studied the relationship between FLC levels and clinical features in 730 patients with newly diagnosed AL. The plasma cell clone was λ in 72% patients, and κ in 28% patients. κ-AL had more GI tract and liver involvement, where as renal involvement was more with λ-AL. While the overall survival (OS) was similar for κ and λ-AL, the median OS for those without an identifiable serum heavy chain was significantly shorter (12.6 vs 29.9 months; P = .02). The OS was shorter among those with a higher dFLC (involved FLC-uninvolved FLC; κ > 29.4 mg/dL or λ > 18.2 mg/dL using median for cutoff); 10.9 vs 37.1 months; P < .001. In multivariate analysis, dFLC was independent of other prognostic factors. The type of light chain impacts the spectrum of organ involvement and the FLC burden correlates with survival in AL.

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Serum immunoglobulin free light-chain measurement in primary amyloidosis: Prognostic value and correlations with clinical features

Abstract

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