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Amyloidosis (Familial Amyloidosis (Investigations (Positive Congo red…
Amyloidosis
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Primary Amyloidosis (AL)
Organs Involved
Nerves: Peripheral + autonomic neuropathy, carpal tunnel syndrome
Gut: macroglossia (big tongue), malabsorption perforation, haemorrhage, obstruction + hepatomegaly
Heart: restrictive cardiomyopathy, arrhythmias, angina
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Pathophysiology
Proliferation of plasma cell clone -> Amyloidogenic monoclonal immunoglobulins -> Fibrillar light chain protein deposition -> Organ failure -> Death
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Definition
Group of disorders characterized by extracellular deposits of a protein in abnormal fibrillar form, resistant to degradation