Characteristics of patients with systemic amyloidosis according to the amyloid protein involved
| . | AL λ . | AL κ . | AA . | Mutated ATTR . | Senile ATTR . | Others* . | P . |
|---|---|---|---|---|---|---|---|
| n (%) | 221 (52) | 99 (24) | 69 (16) | 25 (6) | 5 (1) | 4 (1) | |
| Age, median (range) | 63 (30-87) | 63 (28-86) | 63 (34-89) | 61 (40-79) | 75 (65-84) | 67 (66-70) | |
| Gender (M/F) | 121/100 | 63/36 | 26/43 | 21/4 | 5/0 | 3/1 | <.05 |
| MC by serum and urine IFE + serum FLC (%) | 99.5 | 98.9 | 23.2 | 24 | 0 | 0 | <.001 |
| Organ involvement | |||||||
| Heart (%) | 66.7 | 56.6 | 26.1 | 80 | 100 | 50 | <.001 |
| Urinary proteins >500 mg/24 h (%) | 55.4 | 55.6 | 84 | 0 | 0 | 75 | <.001 |
| Kidney failure (%) | 22.5 | 41.4 | 78.3 | 0 | 0 | 75 | <.001 |
| Liver (%) | 11.7 | 22.2 | 7.2 | 0 | 0 | 50 | |
| GI (%) | 4.5 | 4 | 5.8 | 0 | 0 | 0 | |
| PNS/ANS (%) | 20.3 | 22.2 | 5.8 | 76 | 0 | 0 | <.05 |
| Soft tissue (%) | 30.2 | 24.2 | 0 | 0 | 0 | 0 | <.05 |
| . | AL λ . | AL κ . | AA . | Mutated ATTR . | Senile ATTR . | Others* . | P . |
|---|---|---|---|---|---|---|---|
| n (%) | 221 (52) | 99 (24) | 69 (16) | 25 (6) | 5 (1) | 4 (1) | |
| Age, median (range) | 63 (30-87) | 63 (28-86) | 63 (34-89) | 61 (40-79) | 75 (65-84) | 67 (66-70) | |
| Gender (M/F) | 121/100 | 63/36 | 26/43 | 21/4 | 5/0 | 3/1 | <.05 |
| MC by serum and urine IFE + serum FLC (%) | 99.5 | 98.9 | 23.2 | 24 | 0 | 0 | <.001 |
| Organ involvement | |||||||
| Heart (%) | 66.7 | 56.6 | 26.1 | 80 | 100 | 50 | <.001 |
| Urinary proteins >500 mg/24 h (%) | 55.4 | 55.6 | 84 | 0 | 0 | 75 | <.001 |
| Kidney failure (%) | 22.5 | 41.4 | 78.3 | 0 | 0 | 75 | <.001 |
| Liver (%) | 11.7 | 22.2 | 7.2 | 0 | 0 | 50 | |
| GI (%) | 4.5 | 4 | 5.8 | 0 | 0 | 0 | |
| PNS/ANS (%) | 20.3 | 22.2 | 5.8 | 76 | 0 | 0 | <.05 |
| Soft tissue (%) | 30.2 | 24.2 | 0 | 0 | 0 | 0 | <.05 |
Kidney failure defined by estimated creatinine clearance <60 mL/min.
F, female; GI, gastrointestinal tract; IFE, immunofixation electrophoresis; M, male; MC, monoclonal component; PNS/ANS, peripheral nervous system/autonomic nervous system.
Two cases of mutated apolipoprotein AI were found, 1 with mutated lysozyme and 1 with mutated fibrinogen.