Chronic Myeloid Leukemia Flashcards

1
Q

What is chronic myeloid leukemia

A

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

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2
Q

What is the cause of CML

A

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

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3
Q

What are the 3 phases of leukemia

A

Chronic or indolent phase, advanced phase, blastic phase

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4
Q

What percentage of people with CML are asymptomatic

A

40%

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5
Q

What are specific organ changes that may indicate CML

A

Increased size of spleen and liver

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6
Q

What are the three types of response to theapy for CML

A

Hematologic, cytogenetic, and molecular

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7
Q

What occurs in a hematologic response in CML

A

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

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8
Q

What occurs in a cytogenetic response in CML

A

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

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9
Q

What occurs in molecular response in CML

A

negative PCR result for BCR-ABL mRNA

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10
Q

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

A

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

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11
Q

What is the first drug to be used to treat CML

A

Imatinib

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12
Q

What are the 2nd generation TKIs for CML

A

Dasatinib, Nilotinib, Bosutinib

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13
Q

What is the 3rd generation TKI for CML

A

Ponatinib

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14
Q

What is the MOA of imatinib

A

Blocks ATP from binding BCR-ABL

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15
Q

What are the side effects of imatinib

A

Myelosuppression, diarrhea, musculoskeletal pain, rash, edema

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16
Q

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

A

CYP3A4 and 2C9

17
Q

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

A

Dasatinib and Nilotinib

18
Q

What are the adverse effects of using dasatinib, avoided

A

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

19
Q

What patients should avoid nilotinib

A

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

20
Q

What are the side effects of nilotinib

A

myelosupppresion, qt pronlongation and pancreatitis

21
Q

What are the side effects of using bosutinib

A

diarrhea, nausea, abdominal pain, vomitting

22
Q

Why would a patient be given ponatinib

A

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

23
Q

Which TKIs need to be taken with food

A

Imatinib and Bosutinib

24
Q

Which TKIs can be taken with or without food

A

Dasatinib and Ponatinib

25
Q

Which TKI should be taken on an empty stomach

A

Nilotinib

26
Q

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

A

Bosutinib, Dasatinib, Nilotinib/ Ponatinib

27
Q

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

A

Nilotinib

28
Q

What can be given for a pleural effusion

A

Steroids

29
Q

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

A

All of the 1st and 2nd generation TKIs

30
Q

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

A

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

31
Q

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

A

True

32
Q

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

A

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

33
Q

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

A

3 months, 6 months and after

34
Q

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

A

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

35
Q

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

A

All 2nd and 3rd generation TKIs

36
Q

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

A

True