Table 5 Primary causes of death in the AL amyloidosis cohort.

From: Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Ā 

All deaths

Early deaths (ā‰¤6 months)

Late deaths (>5 years)

Disease-related deaths

Disease-unrelated deathsa

Identified cause, N

1160

354

231

1042

103

ā€ƒOrgan failure (%)

564 (49)

169 (48)

94 (41)

558 (54)

6 (6)

ā€ƒCardiac (%)b

375 (32)

130 (37)

41 (18)

372 (36)

3 (3)

ā€ƒRenal (%)

124 (11)

13 (4)

41 (18)

121 (12)

3 (3)

ā€ƒHepatic (%)

37 (3)

22 (6)

2 (1)

37 (4)

0 (0)

ā€ƒAutonomic (%)

28 (2)

4 (1)

10 (4)

28 (3)

0 (0)

ā€ƒSudden unexpected death (%)c

266 (23)

106 (30)

42 (18)

246 (24)

5 (5)

ā€ƒInfection and/or sepsis (%)

125 (11)

25 (7)

36 (16)

95 (9)

30 (29)

ā€ƒTreatment-related event (%)

49 (4)

16 (5)

9 (4)

39 (4)

10 (10)

ā€ƒMajor vascular event (stroke, MI, VTE) (%)

48 (4)

19 (5)

8 (3)

31 (3)

17 (17)

ā€ƒHemorrhage (%)

33 (3)

12 (3)

10 (4)

29 (3)

4 (4)

ā€ƒMalignancy (%)

21 (2)

1 (<1)

16 (7)

4 (<1)

17 (17)

ā€ƒOther (%)

54 (5)

6 (2)

16 (7)

40 (4)

14 (14)

Unidentified cause, N

500

74

179

7

91

  1. aDeaths occurring while in remission and off plasma cell-directed treatment, or with clear relation to a co-morbid condition. Relation to AL amyloidosis was evaluable for 98 cases despite an unidentified primary cause of death.
  2. bCardiac failure includes 36 (10%) cardiac arrhythmia events.
  3. cThere were 15 patients with sudden death for whom relation to AL amyloidosis was uncertain.
  4. MI myocardial infarction, VTE venous thromboembolism.
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