Primary amyloidosis natural history, complications and prognosis

In primary amyloidosis, insoluble fibrils of AL amyloid are deposited in organs, causing organ dysfunction and eventually death. Patients with primary amyloidosis may eventually suffer from heart failure, nephrotic syndrome, hepatomegaly and peripheral neuropathy. In primary amyloidosis or AL amyloidosis, the survival rate depends upon the type of organ involvement and the hematological response to treatment. In AL amyloidosis, untreated individuals have the worst prognosis. In this group of patients, the median survival is one to two years.

• In primary amyloidosis, insoluble fibrils of AL amyloid are deposited in the organs, causing organ dysfunction and eventually death.^[1]
• In AL amyloidosis, untreated individuals have the worst prognosis. In this group of patients, median survival is one to two years.^[2]
• Patients with amyloidosis may eventually suffer from heart failure, nephrotic syndrome, hepatomegaly and peripheral neuropathy.

In patients with primary amyloidosis, the most frequent complications include:^[3]

• Heart failure
• Nephrotic syndrome
• Hepatomegaly
• Peripheral neuropathy
• Autonomic neuropathy 

• In primary (AL) amyloidosis, survival rate depends on:^[4]
  • Type of organ involvement (amyloid heart disease is the main prognostic factor)
  • The severity of various organ involvement
  • Hematological response to treatment
• The median survival of patients with AL amyloidosis is aproximately 3.8 years.^[5]
• In primary (AL) amyloidosis 27% of the patients die within 1 year of diagnosis.
• The major determinant of outcome in amyloidosis is the extent of cardiac involvement.
  • Cardiac amyloidosis is the cause of death in 75% of the patients who died, including sudden death in 25%.

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