What is the characteristic feature of primary amyloidosis _________________?
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Answer: D. All of the above
Explanation
Primary amyloidosis is a systemic condition associated with specific underlying plasma cell dyscrasias. It frequently involves vital organs such as the heart, skin, and skeletal muscles. A large percentage of affected patients present without any other distinct associated disease. Amyloidosis involves the extracellular deposition of abnormal misfolded fibrillar proteins. Primary amyloidosis specifically features monoclonal immunoglobulin light chain deposition. Plasma cell disorders like multiple myeloma often contribute to systemic amyloid accumulation.
Early diagnosis of systemic amyloidosis is challenging due to diverse and non-specific symptoms. Cardiac involvement in primary amyloidosis can lead to severe restrictive cardiomyopathy and failure. Tissue biopsies of the gingiva or fat pad help confirm suspected amyloid protein deposits. Tissue involvement of the heart and skin is characteristic but represents only one correct aspect. The absence of a distinct pre-existing disease in many cases is also a recognized feature. Plasma cell dyscrasias are present in half of patients, making all listed assertions correct.
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