Congo red staining of abdominal fat has confirmed a diagnosis of Amyloidosis in a 28-year-old man. Which is the most common cause of death in primary AL Amylodosis?
High-Yield Explanation
Cardiac failure is the most common cause of death in primary or AL amyloidosis. Cardiac involvement is seen in 40% of patients with AL amyloidosis. Amyloid involvement of myocardium, intramural coronary aeries and conduction system may cause congestive hea failure, ischemic syndrome and rythm disturbances. Diagnosis of amyloidosis should be considered in patients with unexplained nephropathy, cardiomyopathy (diastolic dysfunction), neuropathy, enteropathy, or pathognomonic soft tissue findings of macroglossia or periorbital ecchymoses. Pathologic diagnosis of amyloid fibrils can be made by congo red staining of aspirated abdominal fat or of an involved organ biopsy specimen.