Clinical features, at time of fat tissue acquisition, of the 26 systemic amyloidosis patients included in the study
Sample . | Amyloid type* . | Age (y), sex . | CR score† . | Amyloid organ involvement‡ . | Serum 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 | 2 |
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 |
Sample . | Amyloid type* . | Age (y), sex . | CR score† . | Amyloid organ involvement‡ . | Serum 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 | 2 |
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.