Prognosis

Immunoglobulin light chain (AL) amyloidosis

The prognosis for patients with AL amyloidosis following treatment is dependent on therapeutic suppression of light chain synthesis. Outcome is also determined by the severity of cardiac involvement.[202]

Survival statistics among 2337 individuals with AL amyloidosis referred to the Boston University Amyloidosis Center have been reported according to date of diagnosis.[203]

  • 1980-1989: 1.4 years overall median survival; 15% 5-year overall survival (OS); 7% 10-year OS

  • 1990-1999: 2.6 years overall median survival; 36% 5-year OS; 18% 10-year OS

  • 2000-2009: 3.3 years overall median survival; 40% 5-year OS; 22% 10-year OS

  • 2010-2019: 4.6 years overall median survival; 48% 5-year OS; non-evaluable 10-year OS.

​Cardiac failure was the leading cause of death (32%). Individuals without cardiac involvement experienced the greatest improvement in overall survival (median OS increasing >6 years over the study period).[203]​ Mortality unrelated to AL amyloidosis increased with time (3% [1980-1989], 8% [1990-1999], 8% [2000-2009], 16% [2010-2019]), and with long-term survival (29% for deaths occurring >10 years after diagnosis).[203]

In another institution, 25% of patients with AL amyloidosis who were diagnosed after 2004 survived for over a decade.[44] Of these long-term survivors, 67% had a complete haematological response and 30% had a very good partial response. Long-term survivors had more limited organ involvement and were less likely to have cardiac, liver, and nerve involvement.

Staging systems may be used to prognosticate survival at diagnosis and at relapse in patients with AL amyloidosis. The European modification of the original Mayo staging system (which utilises a higher threshold for NT-proBNP) may be preferred to other staging criteria because of its discriminative accuracy in detecting patients with the best and worst prognoses, and its applicability in high-risk patients (eGFR <50 or atrial arrhythmia).[204][205]​​ Adding dFLC (difference between the involved to the uninvolved light chain) to a cardiac biomarker-based model increases ability to predict for long-term survival.[204]

Secondary (AA) amyloidosis

The prognosis for patients with AA amyloidosis depends on the control of the underlying disease.

Serum amyloid A (SAA) protein levels are highly predictive of survival.

Suppression of SAA levels to normal (<10 mg/L) is commonly associated with survival in excess of 10 years. However, the relative risk of death increases fivefold for SAA levels between 9 mg/L and 16.6 mg/L, and 17-fold for SAA levels between 87 mg/L and 154 mg/L.[16]

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