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Chapter: Pathology: Amyloidosis

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Amyloidosis: Clinical Features

In systemic forms of amyloidosis, the kidney is the most commonly involved organ, and patients may experience nephrotic syndrome and/or progressive renal failure.

CLINICAL FEATURES

In systemic forms of amyloidosis, the kidney is the most commonly involved organ, and patients may experience nephrotic syndrome and/or progressive renal failure. Cardiac involvement may cause restrictive cardiomyopathy and conduction dis-turbances. Other clinical features include hepatosplenomegaly and involvement of the gastrointestinal tract, which may produce tongue enlargement (macroglossia, primarily in AL type) and malabsorption.

Diagnosis in systemic forms of amyloidosis can be established with biopsy of therectal mucosa, gingiva, or the abdominal fat pad; Congo red stain shows apple green birefringence under polarized light of amyloid deposits. The prognosis of systemic amyloidosis is poor. AL amyloidosis is diagnosed by serum and urinary protein electrophoresis and immunoelectrophoresis. Proteomic analysis is another diag-nostic tool.


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