APML Flashcards
How are patients with APL monitored after consolidation
BMB to document remission after consolidation
PCR
Management of negative PCR after consolidation
maintenance therapy as per initial treatment protocol
management of PCR positive disease after consolidation
Repeat PCR in 2-4 weeks for confirmation and to rule out false positive
IF positive this means relapse
differentiation syndrome clinical features
patient treated with ATRA (usually 10-12 days after starting) + leukocytosis + (dyspnea, fever, pulmonary edema or infiltrates, effusions, weight gain, bone pain)
differentiation syndrome treatment
Dexamethasone 10 mg BID
General urgency
APL is an emergency
Why is ATRA given ASAP?
Prevent bleeding/DIC
Specificity of aeur rods for APML
not specific but suggestive
pathognomonic finding for APML
bilobed nuclei
high risk APML features
WBC greater than 10k
translocation characteristic of APML
t(15;17)
why WBC is considered higher-risk disease in APML
confers a higher risk of differentiation syndrome once ATRA is started
morphologic variants of APML
hypergranular and microgranular
induction therapy for standard risk
ATRA + arsenic trioxide
induction regimens for high risk in general
ATRA + daunorubicin or idarubicin
*bunch of others
arsenic mechanism
Induces apoptosis of leukemic promyelocytes
Immunophenotype of APML
- HLA-DR negative
- CD34 negative
- MPO positive
- CD33 positive
treatment of relapse after CR1
- 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
Primary RF for differentiation syndrome (specific #)
WBC greater than 10K
features of micro or hypogranular subtype on Path
- absence of granules + predominantly bilobed nucleus
microgranular clinical significance
- associated with high white count and rapid doubling time
other impt ADE of ATRA
pseudotumor cerebri
low vs high risk criteria
high risk = WBC over 10K
term for APML with few granules + clinical significance
- hypo or microgranular
- aggressive, high wbc count with rapid doubling time
Risks and benefits of reduced-intensity conditioning (RIC)
- 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
Other term for conventional conditioning
myeloablative conditioning
APML prognosis vs. AML
- short term it is much worse, but long term it is much better (prognosis is excellent long term)
