Amyloidosis

Question 1

What is primary amyloidosis? What is secondary amyloidosis?

Answer 1

AL, Ig light chains coming from a clonal B cell or plasma cell disorder associated with lymphoma or myeloma.
AA, acute phase reactant proteins occurring in the setting of chronic inflammation or infection

Question 2

What tissue is most often biopsied for amyloid?

Answer 2

Abdominal fat

Question 3

What amyloidosis is most common is North America and what age and what is the prognosis usually?

Answer 3

AL
After 40
Can get bad real quick

Question 4

Diagnosis of AL is often made when? What is the first, second and third organs involved? Two pathognomic findings of AL? Two non specific symptoms to think of?

Answer 4

Organs are involved
Kidney then heart (Cardiac arrhythmias), CNS and then obviously several others
Big tongue and racoon eyes
Weight loss and fatigue

Question 5

2 things found for diagnostic purposes of AL?

Answer 5

Light chains or whole Ig in urine

Increased plasma cells in bone marrow

Question 6

2 things we can treat the plasma cell burden with in AL?

Answer 6

Melphalen and prednisone/dexamethasone

Can even try stem cell