SET7

Question 1

How is accelerated CML defined by both MD Anderson and WHO?

Answer 1

>20% Basophils; also these often have 10-19% blasts, whereas Blast phase has >20%

Question 2

How does the Leukocyte Alkaline Phosphatase (LAP) score work?

Answer 2

You basically give a dye (Naphthyl AS-B1) which is hydrolyzed in cytoplasm of neuts and couples with a salt to form a blue pigment; 100 neutrophils are scored from 0 to 4+ and that is the total LAP score; variable scoring is seen in reactive leukocytosis and a low score seen in CML

Question 3

Which TKI in CML can cause pancreatitis and QTc prolongation?

Answer 3

Nilotinib

Question 4

T/F: mutational testing for BCR-ABL is generally done upfront at time of CML diagnosis

Answer 4

False, it is usually done when pt has had some sort of relapse whether cytogenetic, molecular, or hematologic i.e. implying some sort of resistance to the TKI

Question 5

Can men on Imatinib continue it while trying to have kids? Women?

Answer 5

Men yes, women no

Question 6

Which TKI in CML can cause bone pain and periorbital edema?

Answer 6

Imatinib

Question 7

What is the standard of care with respect to continuing vs. discontinuing a BCR-ABL TKI if a patient w/ CML is in CMR (complete molecular remission)?

Answer 7

You always continue it unless the pt is pregnant then it depends on how long pt has been in CMR and whether or not the particular TKI is safe or not (i.e. Imatinib not safe)

Question 8

Blastic Plasmacytoid Dendritic Cell Neoplasm was formerly known as _________

Answer 8

Blastic Natural Killer Cell Lymphoma OR CD4/CD56 Hematodermic Neoplasm

Question 9

What are the AE of ponatinib?

Answer 9

Arterial and Venous Thrombosis; therefore only approved for CML harboring T315I (either chronic, accelerated, or blast phase) or Ph+ ALL harboring T315I

Question 10

What TKI is reasonable for Ph+ ALL harboring a T315I mutation?

Answer 10

Ponatinib

Question 11

What is a major AE of Nilotinib?

Answer 11

QTc prlongation and Pancreatitis

Question 12

Blastic Plasmacytoid Dendritic Cell Neoplasm can sometimes present with indolent cutaneous involvment known as ______

Answer 12

Hematodermic (this is why it was called CD4/CD56 Hematodermic Neoplasm

Question 13

What value of RT-PCR for BCR-ABL defines it as Major Molecular Remission?

Answer 13

<0.2 % (so even if increase from 0.05 to 0.1% that is still MMR)

Question 14

What if there is a cytologic relapse at the 18 month check up after being on TKIs?

Answer 14

Need to switch TKIs and check BCR-ABL mutation status as, depending on the findings, a different TKI may be in order.

Question 15

T/F: sometimes CML blast crises can have lymphoblastic characteristics

Answer 15

True. If the flow shows TdT+ and other lymphoblastic phenotypic characteristics then it is lymphoblastic blast crisis; Treat like a Ph+ ALL

Question 16

What if a pt has CML and there are >20% blasts staining positive for TdT?

Answer 16

It can be an ALL blast crisis, this is possible with CML

Question 17

The DASISION study compared Imatinib vs. Dasatinib as upfront tx for Chronic Phase CML? What was found?

Answer 17

Dasatinib did NOT have improved OS but it did have superior cytogenetic response rate, superior molecular remission rate, and decreased rate of progression to advanced or blast phase

Question 18

If a CML pt goes into blast crisis and obtains allo-HSCT what is the rule for maintenance TKI? How often do you follow BCR-ABL transcript?

Answer 18

They should be on maintenance TKI and you should follow q6 months x3 years

Question 19

What TKIs are approved first line for chronic phase CML? Which are approved 2nd line?

Answer 19

Imatinib, Dasatinib, and Nilotinib; Ponatinib and Bosutinib

Question 20

What is a major AE of Dasatinib?

Answer 20

Pleural Effusions

Question 21

If checking a BCR-ABL mutation status reveals F317L you should use ______

Answer 21

Nilotinib

Question 22

T/F: the dosing of TKIs differs depending on the phase of CML

Answer 22

True. All TKIs are dosed higher in accelerated phase (AP CML) vs. chronic phase

Question 23

What are some AE of Imatinib?

Answer 23

Myalgias/Arthralgias and periorbital edema

Question 24

Is it possible for a pt w/ CML to have cytogenetics not showing t(9;22)?

Answer 24

Yes, so if this is the case you should still send for a BCR-ABL transcript i.e. many are “Ph-“ but carry the BCRABL1 oncoprotein (detected by PCR) and still responsive to TKIs

Question 25

What do you do if someone with CML is failing a TKI?

Answer 25

Switch to another TKI and check BCR-ABL mutation status; UNLESS it is a molecular relapse may be able to increase dose, but if ANY cytological relapse at all need to switch

Question 26

What is the preferred treatment for Blastic Plasmacytoid Dendritic Cell Neoplasm?

Answer 26

An acute leukemia-type regimen followed by allo-HSCT

Question 27

What TKI in CML should be avoided in ppl with CHF?

Answer 27

Dasatinib

Question 28

What TKI can cause pleural effusions?

Answer 28

Dasatinib; avoid in CHF

Question 29

What TKI in CML can cause venous and arterial thromboses?

Answer 29

Ponitinib; only used if T315I BCR-ABL mutation

Question 30

What is an MMR? CCyR?

Answer 30

Major Molecular Remission; Complete Cytological Remission

Question 31

What rare hematologic disease, characterized as an acute leukemia is made of a clonal proliferation of precursors of plasmacytoid dendritic cells?

Answer 31

Blastic Plasmacytoid Dendritic Cell Neoplasm

Question 32

If checking a BCR-ABL mutation status reveals V299L or T315A you should use _____

Answer 32

Nilotinib

Question 33

What do you do if a pt with CML has MMR and the BCR-ABL transcript increases from 0.05 to 0.1%?

Answer 33

Just keep monitoring because <0.2% is still MMR

Question 34

If checking a BCR-ABL mutation status reveals Y253H, E255K, or F359V you should use ______

Answer 34

Dasatinib

Question 35

What is Blastic Plasmacytoid Dendritic Cell Neoplasm?

Answer 35

A rare hematologic dz that acts aggressively and is therefore characterized as an acute leukemia which is made up of proliferation of clonal plasmacytoid dendritic cells

Question 36

What is the significance of T315I in CML?

Answer 36

It is a gatekeeper mutation in which there is substitution of threonine to isoleucine and so Nilotinib and Dasatinib are NOT effective; need Ponatinib DESPITE its arterial and venous thrombosis risk

Question 37

Can Imatinib be used in pregnancy?

Answer 37

No, it can cause fetal malformations

Question 38

What CML TKI can cause bad diarrhea and is approved as 2nd line?

Answer 38

Bosutinib

Question 39

Which TKI is best for CML pts harboring T315I?

Answer 39

Ponatinib; otherwise you hold off on this drug as it causes arterial and venous thromboses

Question 40

What is the Leukocyte alkaline phosphatase score in CML?

Answer 40

Low; high in leukemoid reactions