Table 2 Survival intervals and rates by era of diagnosis.
From: Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study
| Ā | All eras (1980ā2019) | Era 1 (1980ā1989) | Era 2 (1990ā1999) | Era 3 (2000ā2009) | Era 4 (2010ā2019) |
|---|---|---|---|---|---|
All patients | |||||
Deaths, n (%) | 1660 (71) | 182 (98) | 532 (93) | 663 (77) | 283 (40) |
Median age at death, years (IQR) | 66 (58ā73) | 63 (56ā69) | 66 (57ā73) | 66 (58ā73) | 68 (61ā75) |
Median OS, years (95% CI) | 3.0 (2.8ā3.4) | 1.4 (1.0ā1.8) | 2.6 (2.2ā3.0) | 3.3 (2.9ā3.8) | 4.6 (3.8ā5.6) |
6-month mortality rate, % (95% CI) | 16 (15ā18) | 23 (18ā30) | 17 (14ā21) | 17 (14ā19) | 13 (10ā16) |
2-year OS, % (95% CI) | 60 (58ā62) | 41 (34ā48) | 56 (52ā60) | 60 (57ā64) | 71 (67ā75) |
10-year OS, % (95% CI) | 20 (18ā22) | 7 (4ā12) | 18 (15ā21) | 22 (19ā25) | ā |
Subgroups | |||||
āMedian OS, years (95% CI) | |||||
Age ā„70 years | 2.1 (1.8ā2.4) | 0.8 (0.5ā1.6) | 1.8 (1.0ā2.0) | 2.5 (1.8ā3.3) | 2.2 (1.6ā2.6) |
HDM/SCT-treated | 7.6 (6.8ā8.2) | ā | 5.5 (4.5ā6.9) | 8.0 (6.9ā9.2) | 8.8 (8.1āNR) |
Non-SCT-treated | 2.9 (2.6ā3.3) | 1.6 (0.8ā2.3) | 2.6 (2.0ā3.5) | 2.9 (2.3ā3.3) | 3.8 (2.9ā4.8) |
Presence of cardiac involvement | 2.0 (1.6ā2.3) | 0.9 (0.6ā1.3) | 1.4 (0.9ā2.0) | 1.7 (1.4ā2.3) | 2.6 (2.4ā3.5) |
Absence of cardiac involvement | 7.0 (6.1ā7.9) | 2.6 (1.9ā3.2) | 7.2 (5.8ā8.3) | 6.8 (5.9ā8.2) | 8.8 (7.5ā10.1) |