Gen Med

Question 1

Condition

Answer 1

Amyloidosis

Apple green bifringence w/ congo red staining on polarized light microscopy

AA most common (ATTR less so, AA very rare)

Question 2

Alpha 1 antitrypsin def - associations

Answer 2

• COPD
• Liver dis (unless ZZ null phenotype)
• GPA

Question 3

Alpha 1 antitrypsin def - Genotypes

Answer 3

M - normal

Z - null AATD produced in ZZ

AAT is an acute phase protein so measure w/ CRP

Question 4

Rx AL amyloidosis

Answer 4

Dx With SPEP, SFLC, BMAT

Rx: Ø dis modifying agents, even if plasma cell dyscrasia stopped

• Trial chemo (bortezomib-based) + autologous SCTx if fit enough
  
  • Not lenalidomide - poorly tolerated
  • Not HF meds - also poorly tolerated (esp β blockers), high rates of PPM (2⁰ AVB)