APML

Question 1

characteristic translocation

Answer 1

t(15;17)

Question 2

Other translocation that is characteristic of APML

Answer 2

t(11;17)

Question 3

Immunophenotype of APML cells

Answer 3

MPO+, CD33+
CD34-, HLADR-

Question 4

classic boards clinical presentation

Answer 4

younger patient with DIC or intracranial hemorrhage

Question 5

High risk APML criteria (white count threshold)

Answer 5

• WBC >10k

Question 6

high risk APML management

Answer 6

Need chemo, below strategy at UMass (Preferred)
Initial ATRA + anthracycline - idarubicin (ATO differentiates cells, then chemo kills off differentiated cells)
Prophylactic prednisone 1 mg/kg at D1
then ATO starts at D9 w/
*IF high CD33 expression AND not in DIC, Gemtuzumab also an option

Question 7

Starting dose for ATRA

Answer 7

45 mg/m2 in 2 equally PO divided doses

Question 8

ATO monitoring

Answer 8

1) QTc
2) Aggressive K and magnesium supplementation

Question 9

Microgranular variant finding on peripheral smear

Answer 9

bilobed nucleus

Question 10

Management of positive PML/RARA PCR in peripheral blood
*classic board question

Answer 10

• always repeat because can be transiently positive

Question 11

Management of first relapse

Answer 11

ATRA + ATO or single agent GO
*IF obtain CR2, consolidate with auto-SCT

Question 12

management of second relapse in APL

Answer 12

Allo-transplant

Question 13

Platelet count goal during induction in leukemia

Answer 13

10k