Clinical and biochemical characteristics of patients at diagnosis of AL amyloidosis or MM
Code . | Gender, age (y) . | Cardiac stage* . | Diagnosis . | Organs involved† . | Protein source . | Serum λ FLC (mg/L) . | κ/λ FLC ratio . | Proteinuria (g/24 h) . | Creatinine (mg/dL) . | Cardiac parameters . | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Recombinant . | BJ . | Serum . | NT-pro-BNP (ng/L) . | cTnI (ng/mL) . | IVS (mm) . | PW (mm) . | EF (%) . | |||||||||
H-1 | M, 54 | III | AL | H, PNS, ST | x | x | 839 | 0.0018 | 0.98 | 0.74 | 1444 | 0.222 | 16.8 | 16 | 70 | |
H-2 | F, 72 | III | AL | H | x | 769 | 0.02 | 0.23 | 1.58 | 31441 | 0.9 | 16 | 16 | 55 | ||
H-3 | M, 65 | III | AL | H | x | 252 | 0.09 | 0.18 | 1.76 | 4491 | 0.35 | 16 | 16 | 42 | ||
H-4 | M, 46 | II | AL | H | x | 185 | 0.08 | 0.35 | 1.50 | 4942 | 0.08 | 18 | 18 | 40 | ||
H-5 | M, 72 | III | AL | H, K | x | 383 | 0.05 | 8.10 | 2.73 | 21587 | 1.18 | 14.9 | 14.4 | 61 | ||
H-6 | M, 74 | III | AL | H | x | x | 683 | 0.009 | 0.45 | 0.73 | 4300 | 0.2 | 15 | 15 | 42 | |
H-7 | M, 45 | III | AL | H | x | x | 477 | 0.01 | 0.33 | 0.98 | 8882 | 0.16 | 19 | 19 | 45 | |
H-8 | F, 55 | III | AL | H | x | 388 | 0.005 | 0.14 | 0.83 | 5557 | 0.2 | 13 | 14 | 45 | ||
K-1 | M, 69 | I | AL | K | x | 161 | 0.06 | 5.17 | 0.95 | 106 | 0.02 | 13.3 | 13.3 | 63 | ||
K-2 | F, 60 | I | AL | K | x | 104 | 0.06 | 4.76 | 0.58 | 57.8 | 0.001 | 9.6 | 10.4 | 64 | ||
K-3 | M, 68 | I | AL | K | x | 463 | 0.08 | 8.45 | 2.23 | 274 | 0.021 | 11 | 11 | 55 | ||
K-4 | M, 72 | I | AL | K | x | 320 | 0.03 | 6.76 | 0.90 | 230 | 0.022 | 11.5 | 10 | 60 | ||
K-5 | F, 63 | I | AL | K | x | 509 | 0.03 | 2.67 | 0.57 | 40.5 | 0.007 | 9.7 | 9.2 | 61 | ||
K-6 | F, 69 | I | AL | K | x | 228 | 0.08 | 2.33 | 1.39 | 419.5 | 0.005 | 9 | 10 | 60 | ||
ST-1 | F, 55 | I | AL | ST | x | 1220 | 0.0002 | 0.22 | 0.55 | 271 | 0.025 | 11.6 | 11.6 | 57 | ||
MM-1 | F, 73 | MM | — | x | 618 | 0.003 | 0.13‡ | 1.20 | 191 | 0.004 | 10.2 | 10.2 | 55 | |||
MM-2 | F, 71 | MM | — | x | 6130 | 0.001 | 0.52‡ | 2.07 | 42§ | 0.007 | 9 | 9 | 65 | |||
MM-3 | M, 48 | MM | — | x | 573 | 0.011 | 1.87‡ | 0.84 | 14.5 | 0.003 | 10 | 10.5 | 67 | |||
MM-4 | M, 65 | MM | — | x | 1140 | 0.001 | 0.12‡ | 0.89 | 201 | n.a. | 11 | 11 | 65 | |||
MM-5 | M, 37 | MM | — | x | 500 | 0.01 | 0.11‡ | 0.88 | 55 | 0 | 10 | 10 | 64 |
Code . | Gender, age (y) . | Cardiac stage* . | Diagnosis . | Organs involved† . | Protein source . | Serum λ FLC (mg/L) . | κ/λ FLC ratio . | Proteinuria (g/24 h) . | Creatinine (mg/dL) . | Cardiac parameters . | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Recombinant . | BJ . | Serum . | NT-pro-BNP (ng/L) . | cTnI (ng/mL) . | IVS (mm) . | PW (mm) . | EF (%) . | |||||||||
H-1 | M, 54 | III | AL | H, PNS, ST | x | x | 839 | 0.0018 | 0.98 | 0.74 | 1444 | 0.222 | 16.8 | 16 | 70 | |
H-2 | F, 72 | III | AL | H | x | 769 | 0.02 | 0.23 | 1.58 | 31441 | 0.9 | 16 | 16 | 55 | ||
H-3 | M, 65 | III | AL | H | x | 252 | 0.09 | 0.18 | 1.76 | 4491 | 0.35 | 16 | 16 | 42 | ||
H-4 | M, 46 | II | AL | H | x | 185 | 0.08 | 0.35 | 1.50 | 4942 | 0.08 | 18 | 18 | 40 | ||
H-5 | M, 72 | III | AL | H, K | x | 383 | 0.05 | 8.10 | 2.73 | 21587 | 1.18 | 14.9 | 14.4 | 61 | ||
H-6 | M, 74 | III | AL | H | x | x | 683 | 0.009 | 0.45 | 0.73 | 4300 | 0.2 | 15 | 15 | 42 | |
H-7 | M, 45 | III | AL | H | x | x | 477 | 0.01 | 0.33 | 0.98 | 8882 | 0.16 | 19 | 19 | 45 | |
H-8 | F, 55 | III | AL | H | x | 388 | 0.005 | 0.14 | 0.83 | 5557 | 0.2 | 13 | 14 | 45 | ||
K-1 | M, 69 | I | AL | K | x | 161 | 0.06 | 5.17 | 0.95 | 106 | 0.02 | 13.3 | 13.3 | 63 | ||
K-2 | F, 60 | I | AL | K | x | 104 | 0.06 | 4.76 | 0.58 | 57.8 | 0.001 | 9.6 | 10.4 | 64 | ||
K-3 | M, 68 | I | AL | K | x | 463 | 0.08 | 8.45 | 2.23 | 274 | 0.021 | 11 | 11 | 55 | ||
K-4 | M, 72 | I | AL | K | x | 320 | 0.03 | 6.76 | 0.90 | 230 | 0.022 | 11.5 | 10 | 60 | ||
K-5 | F, 63 | I | AL | K | x | 509 | 0.03 | 2.67 | 0.57 | 40.5 | 0.007 | 9.7 | 9.2 | 61 | ||
K-6 | F, 69 | I | AL | K | x | 228 | 0.08 | 2.33 | 1.39 | 419.5 | 0.005 | 9 | 10 | 60 | ||
ST-1 | F, 55 | I | AL | ST | x | 1220 | 0.0002 | 0.22 | 0.55 | 271 | 0.025 | 11.6 | 11.6 | 57 | ||
MM-1 | F, 73 | MM | — | x | 618 | 0.003 | 0.13‡ | 1.20 | 191 | 0.004 | 10.2 | 10.2 | 55 | |||
MM-2 | F, 71 | MM | — | x | 6130 | 0.001 | 0.52‡ | 2.07 | 42§ | 0.007 | 9 | 9 | 65 | |||
MM-3 | M, 48 | MM | — | x | 573 | 0.011 | 1.87‡ | 0.84 | 14.5 | 0.003 | 10 | 10.5 | 67 | |||
MM-4 | M, 65 | MM | — | x | 1140 | 0.001 | 0.12‡ | 0.89 | 201 | n.a. | 11 | 11 | 65 | |||
MM-5 | M, 37 | MM | — | x | 500 | 0.01 | 0.11‡ | 0.88 | 55 | 0 | 10 | 10 | 64 |
Reference ranges: serum λ FLC <26.3 mg/L, κ/λ ratio 0.26 to 1.65, serum creatinine <1.18 mg/dL (men) and <1.02 mg/dL (women), NT-pro-BNP5 <332 ng/L, BNP <50 ng/L, and cTnI <0.04 ng/mL.
BNP, brain natriuretic peptide; cTnI, cardiac troponin I; EF, ejection fraction; F, female; FLC, free light chains; H, heart; IVS, interventricular septum; K, kidney; M, male; n.a., not available; PNS, peripheral nervous system; PW, posterior wall; ST, soft tissues.
According to Gertz et al.15,16
According to the International Consensus Panel criteria.15,16
Entirely constituted by BJ proteins.
BNP (ng/L).