APML

Question 1

How are patients with APL monitored after consolidation

Answer 1

BMB to document remission after consolidation

PCR

Question 2

Management of negative PCR after consolidation

Answer 2

maintenance therapy as per initial treatment protocol

Question 3

management of PCR positive disease after consolidation

Answer 3

Repeat PCR in 2-4 weeks for confirmation and to rule out false positive
IF positive this means relapse

Question 4

differentiation syndrome clinical features

Answer 4

patient treated with ATRA (usually 10-12 days after starting) + leukocytosis + (dyspnea, fever, pulmonary edema or infiltrates, effusions, weight gain, bone pain)

Question 5

differentiation syndrome treatment

Answer 5

Dexamethasone 10 mg BID

Question 6

General urgency

Answer 6

APL is an emergency

Question 7

Why is ATRA given ASAP?

Answer 7

Prevent bleeding/DIC

Question 8

Specificity of aeur rods for APML

Answer 8

not specific but suggestive

Question 9

pathognomonic finding for APML

Answer 9

bilobed nuclei

Question 10

high risk APML features

Answer 10

WBC greater than 10k

Question 11

translocation characteristic of APML

Answer 11

t(15;17)

Question 12

why WBC is considered higher-risk disease in APML

Answer 12

confers a higher risk of differentiation syndrome once ATRA is started

Question 13

morphologic variants of APML

Answer 13

hypergranular and microgranular

Question 14

induction therapy for standard risk

Answer 14

ATRA + arsenic trioxide

Question 15

induction regimens for high risk in general

Answer 15

ATRA + daunorubicin or idarubicin

*bunch of others

Question 16

arsenic mechanism

Answer 16

Induces apoptosis of leukemic promyelocytes

Question 17

Immunophenotype of APML

Answer 17

• HLA-DR negative
• CD34 negative
• MPO positive
• CD33 positive

Question 18

treatment of relapse after CR1

Answer 18

• Given 6 months out from treatment and if previously CR, arsenic trioxide 0.15 mg/kg IV +/- ATRA 45 mg/m2
• Given less than 6 months from treatment with ATRA and ATO, plan for anthracycline-based regimen

Question 19

Primary RF for differentiation syndrome (specific #)

Answer 19

WBC greater than 10K

Question 20

features of micro or hypogranular subtype on Path

Answer 20

• absence of granules + predominantly bilobed nucleus

Question 21

microgranular clinical significance

Answer 21

• associated with high white count and rapid doubling time

Question 22

other impt ADE of ATRA

Answer 22

pseudotumor cerebri

Question 23

low vs high risk criteria

Answer 23

high risk = WBC over 10K

Question 24

term for APML with few granules + clinical significance

Answer 24

• hypo or microgranular

- aggressive, high wbc count with rapid doubling time

Question 25

Risks and benefits of reduced-intensity conditioning (RIC)

Answer 25

• less transplant-related morbidity and mortality, but this is counterbalanced by increased relapse rates. There have been 2 trials comparing RIC to myeloablative conditioning, and they failed to establish which is preferrable

Question 26

Other term for conventional conditioning

Answer 26

myeloablative conditioning

Question 27

APML prognosis vs. AML

Answer 27

• short term it is much worse, but long term it is much better (prognosis is excellent long term)