ALL

Question 1

Poor risk cytogenetics in adult ALL

Answer 1

t(9;22)
KMT2A 
t(8;14)
Complex
Low hypodiploid (30-39 chr)

Question 2

Low hypodiploid ALL is associated with

Answer 2

TP53 mutations

Poor prognosis

Question 3

In children low hypodiploid ALL can be associated with germline TP53 and a manifestation of what syndrome?

Answer 3

Li-Fraumeni syndrome

Question 4

Good risk in adult ALL?

Answer 4

Hyperdiploid

ETV6-RUNX1

Question 5

4 methods to assess for MRD in ALL

Answer 5

High throughput NGS
Flow cytometry
PCR for specific genes (BCR/ABL)
PCR immunoglobulin/TCR genes

Question 6

Trial name and outcome for blinatumomab in MRD positive ALL

Answer 6

BLAST trial. Continuous CR in 74% of blina followed by HSCT compared to 25% in blina alone (no chemo or HSCT)

Question 7

What trumps all other prognostic factors in ALL?

Answer 7

Presence/amount of minimal residual disease

Question 8

Commonly used regimes for adult ALL?

Answer 8

CALGB (cyclophosphamide, asparaginase, daunorubicin, vincristine, prednisone)
Hyper CVAD (no asparaginase)

Question 9

Success rate / CR rate in (CALGB) for ALL in young adults?

Answer 9

85% CR

46% in continuous CR after 3 years

Question 10

Which ALL patients should go to transplant?

Answer 10

Ph+
MLL (all translocations, t4;11 is the worst)
Hypodiploid (less than 44 chromosomes, TP53 pos)
ETP
MRD positive

Question 11

Blinatumomab mechanism

Answer 11

BiTE antibody designed to direct cytotoxic T cells to CD19 expressing cancer cells

Question 12

TOWER study description and outcome

Answer 12

Patients with R/R pre BALL randomized to blinatumomab vs SOC. Chemo. 78% of responders with blina and 48% with SOC achieved MRD negativity. Median OS blina 7.7 months, SOC chemo 4.0 months.

Question 13

Inotuzumab mechanism

Answer 13

Humanized IgG4 antiCD22 monoclonal antibody bound to a toxic natural product calicheamicin. The drug is internalized by endocytosis into cytoplasmic of cd22 pos cells, then calicheamicin moves into the nucleus, binds to minor DNA groove, and causes a break in DNA and cell cycle arrest

Question 14

Trial name and outcome of inotuzumab vs SOC in R/R ALL

Answer 14

INO-VATE. 80.7% vs 29.4% CR, 75% vs 28% MRD.

Better 2 year OS with INO (23%) vs 10% with standard of care

Question 15

Mechanism of action of nelarabine

Answer 15

Prodrug of AraG which accumulates in T lymphoblasts

Question 16

Kymriah generic name

Answer 16

Tisagenlecleucel

Question 17

Significance of CRLF2 in ALL?

Answer 17

Associated with Ph like disease, and has a poorer prognosis with increased risk of relapse