Chronic Myeloid Leukemia

Question 1

What is chronic myeloid leukemia

Answer 1

stem cell disease characterized by excessive accumulation of clonal myeloid (precursor) cells in hematopoietic tissues

Question 2

What is the cause of CML

Answer 2

Translocation between chromosme 9 and 12 causing a fusion of BCR-ABL

Question 3

What are the 3 phases of leukemia

Answer 3

Chronic or indolent phase, advanced phase, blastic phase

Question 4

What percentage of people with CML are asymptomatic

Answer 4

40%

Question 5

What are specific organ changes that may indicate CML

Answer 5

Increased size of spleen and liver

Question 6

What are the three types of response to theapy for CML

Answer 6

Hematologic, cytogenetic, and molecular

Question 7

What occurs in a hematologic response in CML

Answer 7

Normalization of peripheral blood counts (WBC less than 10k, platelets less than 450k, non-palpable spleen)

Question 8

What occurs in a cytogenetic response in CML

Answer 8

Percentage of cells positive for the Philadelphia chromosome in a bone marrow biopsy (elimination of ph-positive cells)

Question 9

What occurs in molecular response in CML

Answer 9

negative PCR result for BCR-ABL mRNA

Question 10

What is the only performed cure of CML, when is it done

Answer 10

Allogenic bone marrow transplantation or stem cell transplantation/ Chronic stage (best in young patients)

Question 11

What is the first drug to be used to treat CML

Answer 11

Imatinib

Question 12

What are the 2nd generation TKIs for CML

Answer 12

Dasatinib, Nilotinib, Bosutinib

Question 13

What is the 3rd generation TKI for CML

Answer 13

Ponatinib

Question 14

What is the MOA of imatinib

Answer 14

Blocks ATP from binding BCR-ABL

Question 15

What are the side effects of imatinib

Answer 15

Myelosuppression, diarrhea, musculoskeletal pain, rash, edema

Question 16

What are the enzyme that most if not all TKIs must be aware of

Answer 16

CYP3A4 and 2C9

Question 17

What are the 2nd generation TKIs that are known to cause QT prolongation

Answer 17

Dasatinib and Nilotinib

Question 18

What are the adverse effects of using dasatinib, avoided

Answer 18

pleural effusions, myelosuppresion, pulmonary hypertension/ history of lung disease

Question 19

What patients should avoid nilotinib

Answer 19

vaso-occlusive vascular disease, ischemic heart disease, peripheral artery occlusive disease, diabetes, pancreatitis

Question 20

What are the side effects of nilotinib

Answer 20

myelosupppresion, qt pronlongation and pancreatitis

Question 21

What are the side effects of using bosutinib

Answer 21

diarrhea, nausea, abdominal pain, vomitting

Question 22

Why would a patient be given ponatinib

Answer 22

Overcome resistance to other TKIs due to BCR-ABLs mutations (T315I)

Question 23

Which TKIs need to be taken with food

Answer 23

Imatinib and Bosutinib

Question 24

Which TKIs can be taken with or without food

Answer 24

Dasatinib and Ponatinib

Question 25

Which TKI should be taken on an empty stomach

Answer 25

Nilotinib

Question 26

What are the TKIs that are most effected by PPIs antacids and H2RAs due to lessened exposure, only affected by PPIs

Answer 26

Bosutinib, Dasatinib, Nilotinib/ Ponatinib

Question 27

Which TKI should not be used in patients with cardiovascular problems because QT prolongation arrhythmia risk, contraindicated with flouroquinolones

Answer 27

Nilotinib

Question 28

What can be given for a pleural effusion

Answer 28

Steroids

Question 29

If a patient has a low risk score chronic CML what are the primary treatment options

Answer 29

All of the 1st and 2nd generation TKIs

Question 30

If a patient has a intermediate or high risk score for chronic CML what are the primary treatment options

Answer 30

All of the 2nd generation TKIs (Bosutinib, Dasatinib, Nilotinib)

Question 31

T/F: If a patient has less than 1% BCR-ABL cells they have TKI sensitive disease no matter the time frame

Answer 31

True

Question 32

If a patient has between 1 to 10% BCR-ABL cells at what time is there disease still considered TKI sensitive, possibly TKI resistant, TKI resistant

Answer 32

3 to 6 months, 12 months, greater than 15 months

Question 33

If a patient has greater than 10% BCR-ABL cells when would it be consider that there possibly has TKI resistant, TKI resistant

Answer 33

3 months, 6 months and after

Question 34

What are the options if a patient possibly has TKI Resistance/ has TKI resistance

Answer 34

Switch to alternate TKI OR continue same TKI (as long as its not imatinib) OR dose escalation (imatinib:800 mg) AND consider evaluation for allogenic HCT/ switch to alternate TKI AND evaluate for allogenic HCT

Question 35

If a patient has advanced ACCELERATED phase CML what is the preferred treatment

Answer 35

All 2nd and 3rd generation TKIs

Question 36

T/F: If a patient is in advanced BLAST phase CML it should be treated as acute leukemia

Answer 36

True