Cytogenetics & Mutations
Therapy Time
Diagnostic Dilemma
Pouttpouri
Relapsed Refractory
100

This cytogenetic abnormality defines APL.

What is t(15;17) PML-RARA?

100

What regimen is considered “7+3”?

Cytarabine for 7 days + Anthracycline for 3 days

100

What percentage of blasts is required for an AML diagnosis (excluding defining translocations)?

What is ≥20% blasts?

100

What is the first-line management for APL differentiation syndrome?

Hold ATRA and give Dexamethasone 10 mg IV BID

Happens 7-10 days after therapy

100

30% of patients will acquire new mutations they didn’t have before (FLT3) and can also lose FLT3 mutations.

What is True?

200

Prognostic implication of NPM1 mutation without FLT3-ITD.

What is favorable risk?

200

The VIALE-A trial established this regimen as standard for unfit AML?

What is AZA/VEN?

(VIALE C also approved LDAC/VEN)

LDAC/Glasdegib also approved

200

What type of granule and rod-like inclusion is seen in Auer rods?

What are Myeloperoxidase-positive primary granules?

200

What infection prophylaxis is recommended during prolonged neutropenia?

What is Antibacterial (FQ), antifungal (azoles), antiviral (acyclovir)?

Need to dose reduce venetoclax if on CYP450 inhibitor (i.e. vori/posaconazole, ciprofloxacin

200

T/F One should pursue transplant in relapsed setting if CR2 has been achieved regardless of orginal risk status

What is T?

300

Therapy related AML Mutations as well as how long they occur after each therapy

Anthracyclines - topo II (11q23) - MLL/KM2TA rearrangement; aggressive; chemorefractory; happens 1-3 years after 

Radiation/alkylating like cytoxan - 5 and -7; 5-7 years after radiation/chemotherapy

300

This is the standard treatment for therapy related AML?

What is VYXEOS (CPX-351)? COMBO/LIPOSOMAL daunorubicin/cytarabine

300

What will flow cytometry show for APML?

What are MPO+, CD33+, CD34-, HLA-DR-?

300

What is the WBC count needed to start venetoclax?

What is < 25K? 

Can use hydrea to bring counts down

300

FLAG IDA is a regimen used in the relapsed setting. It stands for...

What is fludarabine, cytarabine, GCSF, idarubicin?

400

Which mutation confers poor prognosis and resistance to venetoclax?

What is TP53?

400

Which FLT3 inhibitor is used in combination with 7+3 induction in newly diagnosed AML with both ITD/TKD mutations?

What is Midostaurin? (RATIFY trial)

Quizartinib only for ITD

400

What constitutes high risk APML?

Low risk - white count is less than 10K and platelet > 40K

Intermediate - WBC < 10K platelet < 40K

High risk - WBC > 10K

Add gemtuzumab or idarubicin to ATRA/ARSENIC for high risk 

400

These leukemic patients need an LP.

Over 30K (maybe 50K?)

Monocytic Leuekmia (M4/M5)

FLT3 - typically bc of high white count

WAIT until blasts from peripheral blood come down - start chemo, wait for it to come down, then do LP (if platelet too low, then do LP after)

400

This FLT3 inhibitor can be used in relapsed AML (both ITD/TKD)

What is gilterinib?

500

These are the two Core Binding Factor Mutations 

What are t(8;21)(q22;q22) → RUNX1::RUNX1T1 (AML1::ETO)


inv(16)(p13q22) or t(16;16)(p13;q22) → CBFB::MYH11?

500

Which IDH1 and IDH2 inhibitors are FDA approved for AML?

What are Ivosidenib (IDH1) and Enasidenib (IDH2)

500

This is considered unfit for transplant?

What is the Ferrara Criteria?

Age > 75 years old

CHF requiring treatment or EF < 50%

DLCO < 65% or FEV1 < 65%

ECOG 2-3 (most subjective), can be disease dependent 

500

What are two specific things to monitor for patients on while on HIDAC?

What are Cerebellar toxicity and Ocular toxicity (need eye drops)
500

This novel menin inhibitor targets KMT2A-r and NPM1-mutated R/R AML

What is revumenib (or ziftomenib)?