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.
authors.affiliation: Division of Hematology and Internal Medicine, Department of Laboratory Medicine and Pathology, Department of Biochemistry, Mayo Clinic, Rochester, MN, USA. kumar.shaji@mayo.edu
authors.forename: Shaji
authors.initials: S
authors.lastname: Kumar
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Katzmann
authors.forename: Dirk R
authors.initials: DR
authors.lastname: Larson
authors.forename: Colin L
authors.initials: CL
authors.lastname: Colby
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
authors.forename: Suzanne R
authors.initials: SR
authors.lastname: Hayman
authors.forename: Francis K
authors.initials: FK
authors.lastname: Buadi
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
authors.forename: Steve R
authors.initials: SR
authors.lastname: Zeldenrust
authors.forename: Marina
authors.initials: M
authors.lastname: Ramirez-Alvarado
authors.forename: Raynell J
authors.initials: RJ
authors.lastname: Clark
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.forename: S Vincent
authors.initials: SV
authors.lastname: Rajkumar
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000453:epidemiology / Q000401:mortality / Q000473:pathology; D005260:Female; D006331:Heart Diseases; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D000075363:Immunoglobulin Light-chain Amyloidosis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D007674:Kidney Diseases; D008107:Liver Diseases; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology; D011379:Prognosis; D016019:Survival Analysis
chemical_list: D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains
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