Amyloidosis Flashcards
What is amyloidosis?
A disorder of protein metabolism
What is amyloid protein?
Beta pleated sheets, resistant to breakdown and insoluble
What are the types of amyloidosis?
Primary, familial/secondary
What is primary amyloidosis?
Due to proliferating plasma cells producing amyloidogenic immunoglobulins
Where is amyloid deposited in primary amyloidosis?
Kidney, heart, nerves, vessels, gut
What is secondary amyloidosis?
Due to conversion of serum amyloid A to amyloid in kidney, liver, spleen
What do symptoms depend upon?
Affected organ
What are potential symptoms?
Proteinuria/nephrotic syndrome, cardiomyopathy/arrhythmia/angina, neuropathy/carpal tunnel, malabsorption/perforation/obstruction, purpura/bleeding, hepatosplenomegaly
Which form of amyloidosis is fatal?
Primary
How do you diagnose amyloidosis?
Biopsy, Congo red stain under polarised light shows red-green birefringence
How do you treat primary amyloidosis?
Melphalan (chemo) and stem cell transplant, dexamethasone
How do you treat secondary amyloidosis?
Manage underlying condition
How do you treat familial amyloidosis?
Liver transplant
What is seen on a blood film?
Howell-Jolly bodies
