Incidence and Epidemiology of Primary Systemic Amyloidosis (AL) in Olmsted County, Minnesota: 1950 Through 1989

Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis

“…4 Two-thirds of AL patients present with one or two major organ systems involved (heart, kidneys, liver/ GI tract, peripheral nervous system and soft tissues) while a third of patients presents with more than two systems involved. 2 Symptoms reflect involved organ disease. Both the clonal plasma cells and the pathologic light chain proteins are targets for therapy in AL.…”

“…1,2 Although there have been recent advances in AL, there is a major need for a framework for clinical research to encourage the development of new therapies. Access to novel agents and their optimal and safe use are challenges for patients with this rare disease.…”

Epidemiology of AL amyloidosis: a real-world study using US claims data

“…1 The most common and severe type of systemic amyloidosis is amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis. In AL amyloidosis, amyloid fibrils are derived from k or l monoclonal light chains, which are synthesized by a clonal population of plasma cells in the bone marrow.…”

Amyloidosis

“…In this study, the overall age-adjusted and sexadjusted 95% confidence interval was 5.1 to 12.8 per million patients per year. 22 In Amyloidosis a recent Swedish study, the estimated incidence of AL amyloidosis was 3.2 per million per year and AA amyloidosis 2.0 per million per year. 23 The median age for AL and AA amyloidosis is between 55 and 60 years.…”