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 (1980–2019)

Era 1 (1980–1989)

Era 2 (1990–1999)

Era 3 (2000–2009)

Era 4 (2010–2019)

All patients

Deaths, n (%)

1660 (71)

182 (98)

532 (93)

663 (77)

283 (40)

Median age at death, years (IQR)

66 (58–73)

63 (56–69)

66 (57–73)

66 (58–73)

68 (61–75)

Median OS, years (95% CI)

3.0 (2.8–3.4)

1.4 (1.0–1.8)

2.6 (2.2–3.0)

3.3 (2.9–3.8)

4.6 (3.8–5.6)

6-month mortality rate, % (95% CI)

16 (15–18)

23 (18–30)

17 (14–21)

17 (14–19)

13 (10–16)

2-year OS, % (95% CI)

60 (58–62)

41 (34–48)

56 (52–60)

60 (57–64)

71 (67–75)

10-year OS, % (95% CI)

20 (18–22)

7 (4–12)

18 (15–21)

22 (19–25)

–

Subgroups

ā€ƒMedian OS, years (95% CI)

Age ≄70 years

2.1 (1.8–2.4)

0.8 (0.5–1.6)

1.8 (1.0–2.0)

2.5 (1.8–3.3)

2.2 (1.6–2.6)

HDM/SCT-treated

7.6 (6.8–8.2)

–

5.5 (4.5–6.9)

8.0 (6.9–9.2)

8.8 (8.1–NR)

Non-SCT-treated

2.9 (2.6–3.3)

1.6 (0.8–2.3)

2.6 (2.0–3.5)

2.9 (2.3–3.3)

3.8 (2.9–4.8)

Presence of cardiac involvement

2.0 (1.6–2.3)

0.9 (0.6–1.3)

1.4 (0.9–2.0)

1.7 (1.4–2.3)

2.6 (2.4–3.5)

Absence of cardiac involvement

7.0 (6.1–7.9)

2.6 (1.9–3.2)

7.2 (5.8–8.3)

6.8 (5.9–8.2)

8.8 (7.5–10.1)

  1. IQR interquartile range, OS overall survival, CI confidence interval, NR not reached.