CML facts

Question 1

Nilotinib SE

Answer 1

CV
Metabolic syndrome
Pancreatic enzymes/Pnacreatitis
Hyperbilirubinemia
QT prolongation

Question 2

Nilotinib 1st line in CML

Answer 2

ENESTnd

Question 3

Dasatinib 1st line in CML

Answer 3

DASISON

Question 4

Bosutinib 1st line in CML

Answer 4

BEFORE

Question 5

Dasatinib dose

Answer 5

100 mg qd

Question 6

Nilotinib dose

Answer 6

300 mg bid
without food

Question 7

Bosutinib dose

Answer 7

400 mg qd
start gradually
ramp up of 200 for 1-2 weeks
300 for 1-2 weeks

Question 8

Imatinib trial in CML

Answer 8

IRIS

Question 9

Imatinib dose

Answer 9

400 mg qd

Question 10

Imatinib SE

Answer 10

Neutropenia
Fatigue
GI
Rash
Edema
Muscle cramps

Question 11

Goals of Tx in CML

Answer 11

OS
Minimal damage to QOL
Decrease risk of long term toxicity
DMR to allow TFR

Question 12

Dasatinib SE

Answer 12

Neutropenia
Thrombocytopenia and platelet dysfunction
PHTN
Pleural effusion

Question 13

Dasatinib 2nd line in CML

Answer 13

START-R
Phase II
Imatinib resistant
Compared to high dose imatinib
Deeper and longer responses

Question 14

Ponatinib 2nd/3rd line in CML

Answer 14

PACE
phase II
Long term OS 70%
30% ATE

Question 15

Ponatinib 1st line in CML

Answer 15

EPIC- negative trial due to toxicity

Question 16

Ponatinib SE

Answer 16

CV- 26%
Thrombocytopenia
Rash
Dry skin
Abdominal pain

Question 17

Ponatinib dose

Answer 17

45 mg/30mg
reduce to 15 mg when in CCyR
based on OPTIC trial

Question 18

Tx of AP CML

Answer 18

Entity does not exist anymore
Historically
2nd gen are preferred with higher doses

Question 19

BP phenotype in CML

Answer 19

50% myeloid, 33% lymphoblastic, undifferentiated and MPAL -rest

Question 20

Poor risk predictors in BP CML

Answer 20

ACA and >50% blasts
LDH
Thrombocytopenia
Prior TKI
Age > 58
Chromosome 15 aberrations

Question 21

Tx of BP CML

Answer 21

2nd gen TKI followed by allo transplant

Question 22

TFR requirements in CML

Answer 22

CP CML
1st line or 2nd line if intolerance
5 years of TKI (4 years for 2nd gen)
DMR> 2 years (preferred 3 years for MR4)

Question 23

% of ACAs in CML

Answer 23

10-15%

Question 24

Most common ACAs in CML

Answer 24

trisomy 8
additional loss on 22q

Poor prognosis-
isochromosome 17
del 7
3q26

Question 25

Chromosome location of BCR:ABL1

Answer 25

BCR chro 22
ABL1 chro 9

Question 26

% of pts with BCR:ABL1 variants in CML

Answer 26

5%

Question 27

% of pts negative to Ph chromsome on cytogentics in CML

Answer 27

2-5%
still PCR and FISH positive

Question 28

Most common transcripts in CML

Answer 28

e13a2
e14a2
both p210

Question 29

Non p210 BCR:ABL1 transcripts in CML

Answer 29

e1a2/e1a3
resulting in p190

poor prognosis

Question 30

Dasatinib 50 vs 100mg in CML

Answer 30

Similar effectiveness with better toxicity profile

Question 31

Asciminib 2nd line in CML

Answer 31

ASCEMBL trial
compered to bosutinib
assessed MMR at 6 mon which is not an acceptable endpoint
no PFS or OS advantage

Question 32

Asciminib dosage

Answer 32

40 mg bid
200 mg bid if T315I

Question 33

High risk mutations in CML

Answer 33

ASXL1
IKZF1
RUNX1

AIR

Question 33

CML-BP response to Tx

Answer 33

TKI+ Chemo
40% for myeloid
70% for lymphoid

Question 34

Variant translocations in CML %

Answer 34

10-15%
22 and other chromosomes

Question 35

ELTS

Answer 35

Age (less significant than in SOKAL)
Blasts
Splenomegaly
PLT

Question 36

Need for testing NGS and TK mutations in CML at diagnosis

Answer 36

Only in BP
No need in CP

Question 37

2nd gen vs. Imatinib TKIs in CML

Answer 37

Faster and deeper responses
No OS benefit
Different SE

Question 38

Bosutinib SE

Answer 38

GI
Fluid retention
Hepatic
Renal

Question 39

% of mutation of TKI in CML that is significant

Answer 39

> 15%