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Amyloidosis refers to an extremely rare group of disorders caused by extracellular deposition of abnormal fibrillary proteins (-fibrils). This condition can either occur locally or systemically. In systemic AL amyloidosis (immunoglobulin light chain amyloidosis), the defective bone marrow plasma cells produce monoclonal antibodies, which lead to amyloid deposits causing damage in multiple organs. Depending on the manifestation, clinical symptoms vary significantly and can lead to cardiac insufficiency and renal failure. Additionally, an involvement of lung, muscles, and soft tissues may occur Systemic AL amyloidosis is the most common subtype of amyloidosis. Another rare subtype is the localized amyloidosis, which can occur in the upper and lower airways (larynx, trachea and bronchial system), skin, and urinary tract. There are only a few reports on localized amyloidosis of the nasopharynx and none for the Eustachian tube. The derived proteins are only produced in the defected organ itself in this subtype A transition into a systemic disease is very unlikely. A 20-year follow-up of 20 patients has not revealed a single case of transition
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Lauer et al. (2018) studied this question.
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