Amyloidosis can be categorized into localized and systemic disease, depending on the source and origin of the protein as well as if there is spread of the disease; classification is now determined by the type of protein deposits. Systemic disease is more common and consists of primary (AL) ...
Primary amyloidosis has a variable course, but is generally associated with a short life expectancy. To date, no specific therapy has been available. Fifty-three patients with AL amyloidosis seen between 1976 and 1983 were treated with colchicine, and their clinical course and survival were compared...
Pulmonary amyloidosis often occurs as a part of systemic AL (amyloid light chain) amyloidosis. Localized tracheobronchial amyloidosis is a very rare entity. Most often, it is asymptomatic, or if symptomatic, it usually presents with vague symptoms such as cough, dyspnoea, hoarseness, wheezing, ...
The currently approved treatments in AL amyloidosis have the underlying clone as their target, with the aim to reduce the production and, thus, the blood concentration of the amyloidogenic LC [1,2,7,8,9]. Lowering serum monoclonal free LCs can halt the progression of the disease and has a...