What factors are prognostic in systemic AL amyloidosis?
The overall median survival for systemic AL amyloidosis is 12 to 15 months. The most common cause of death is attributable to cardiac involvement. The presence of multiple myeloma reduces the median survival significantly. Grouping of patients by clinical symptoms (heart failure, nephrotic syndrome, peripheral neuropathy, and others) is a useful guide to long-term prognosis. The presence of heart failure is associated with the worst prognosis (median survival, 6 months). The best prognosis is in patients with peripheral neuropathy when it occurs as the sole manifestation (median survival, 56 months). Although the 24-hour urinary total protein excretion does not affect survival, the presence of an increased serum creatinine and failure of serum/urine free light chains to decrease with therapy are poor prognostic signs.
