Table 1

Clinical features, at time of fat tissue acquisition, of the 26 systemic amyloidosis patients included in the study

SampleAmyloid type*Age (y), sexCR scoreAmyloid organ involvementSerum MC (HR-IFE)κ/λ ratio§
P1 AL-λ 61, F 3+ Kidney IgGλ + λ-FLC 0.28 
P2 AL-λ 53, M 3+ Heart, kidney λ-FLC 0.05 
P3 AL-λ 67, M 3+ Heart IgGλ + λ-FLC 0.005 
P4 AL-λ 59, F 3+ Heart, kidney, PNS, ANS λ-FLC 0.06 
P5 AL-λ 64, M 3+ Heart, kidney IgAλ 0.009 
P6 AL-λ 64, M 3+ Heart λ-FLC 0.06 
P7 ATTR (Val30Met) 68, M 3+ PNS  0.69 
P8 AL-λ 68, F 3+ Heart, kidney, liver λ-FLC 0.06 
P9 AL-λ 58, M 3+ Heart, soft tissues λ-FLC 0.03 
P10 AL-λ 67, F 3+ Heart, kidney IgAλ + λ-FLC 0.05 
P11 AL-λ 73, F 3+ Heart, kidney, PNS λ-FLC 0.05 
P12 AL-λ 59, M 4+ Heart, kidney, PNS, ANS λ-FLC 0.02 
P13 AL-λ 70, F 4+ Kidney IgMλ + λ-FLC 0.08 
P14 AL-κ 75, M 4+ Heart, kidney, liver κ- FLC + faint λ-FLC 7.7 
P15 AL-κ 60, M 3+ Heart, soft tissues κ-FLC + faint IgMλ 158 
P16 ATTR (Tyr78Phe) 66, M 3+ Heart  0.64 
P17 AA 70, F 3+ Kidney  0.48 
P18 ATTR (Leu68Ile) 76, M 3+ Heart, PNS  1.31 
P19 ATTR (Ser50Arg) 37, M 3+ Heart, PNS, ANS IgAκ 
P20 ATTR (Ser50Arg) 55, F 3+ Heart, PNS, ANS  0.94 
P21 AA 70, M 4+ Kidney  1.08 
P22 AA 75, F 4+ Kidney  1.4 
P23 AA 66, F 4+ Kidney  1.33 
P24 AA 79, M 3+ Kidney  1.62 
P25 AL-κ 80, M 4+ Heart, soft tissues, PNS IgGκ + κ-FLC 22.3 
P26 AL-κ 63, M 4+ Kidney, PNS IgGκ 21.8 
SampleAmyloid type*Age (y), sexCR scoreAmyloid organ involvementSerum MC (HR-IFE)κ/λ ratio§
P1 AL-λ 61, F 3+ Kidney IgGλ + λ-FLC 0.28 
P2 AL-λ 53, M 3+ Heart, kidney λ-FLC 0.05 
P3 AL-λ 67, M 3+ Heart IgGλ + λ-FLC 0.005 
P4 AL-λ 59, F 3+ Heart, kidney, PNS, ANS λ-FLC 0.06 
P5 AL-λ 64, M 3+ Heart, kidney IgAλ 0.009 
P6 AL-λ 64, M 3+ Heart λ-FLC 0.06 
P7 ATTR (Val30Met) 68, M 3+ PNS  0.69 
P8 AL-λ 68, F 3+ Heart, kidney, liver λ-FLC 0.06 
P9 AL-λ 58, M 3+ Heart, soft tissues λ-FLC 0.03 
P10 AL-λ 67, F 3+ Heart, kidney IgAλ + λ-FLC 0.05 
P11 AL-λ 73, F 3+ Heart, kidney, PNS λ-FLC 0.05 
P12 AL-λ 59, M 4+ Heart, kidney, PNS, ANS λ-FLC 0.02 
P13 AL-λ 70, F 4+ Kidney IgMλ + λ-FLC 0.08 
P14 AL-κ 75, M 4+ Heart, kidney, liver κ- FLC + faint λ-FLC 7.7 
P15 AL-κ 60, M 3+ Heart, soft tissues κ-FLC + faint IgMλ 158 
P16 ATTR (Tyr78Phe) 66, M 3+ Heart  0.64 
P17 AA 70, F 3+ Kidney  0.48 
P18 ATTR (Leu68Ile) 76, M 3+ Heart, PNS  1.31 
P19 ATTR (Ser50Arg) 37, M 3+ Heart, PNS, ANS IgAκ 
P20 ATTR (Ser50Arg) 55, F 3+ Heart, PNS, ANS  0.94 
P21 AA 70, M 4+ Kidney  1.08 
P22 AA 75, F 4+ Kidney  1.4 
P23 AA 66, F 4+ Kidney  1.33 
P24 AA 79, M 3+ Kidney  1.62 
P25 AL-κ 80, M 4+ Heart, soft tissues, PNS IgGκ + κ-FLC 22.3 
P26 AL-κ 63, M 4+ Kidney, PNS IgGκ 21.8 

CR indicates Congo red; MC, monoclonal component; HR-IFE, high-resolution electrophoresis and immunofixation; FLC, free light chains; PNS, peripheral nervous system; ANS, autonomic nervous system; LC-κ, immunoglobulin light chain-κ; and LC-λ, immunoglobulin light chain-λ.

*

Confirmed by IEM.

Graded on subcutaneous fat tissue.11 

Defined according to the International Consensus Panel criteria.12 

§

Reference interval: 0.26-1.65.