SET7 Flashcards
How is accelerated CML defined by both MD Anderson and WHO?
>20% Basophils; also these often have 10-19% blasts, whereas Blast phase has >20%
How does the Leukocyte Alkaline Phosphatase (LAP) score work?
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
Which TKI in CML can cause pancreatitis and QTc prolongation?
Nilotinib
T/F: mutational testing for BCR-ABL is generally done upfront at time of CML diagnosis
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
Can men on Imatinib continue it while trying to have kids? Women?
Men yes, women no
Which TKI in CML can cause bone pain and periorbital edema?
Imatinib
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)?
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)
Blastic Plasmacytoid Dendritic Cell Neoplasm was formerly known as _________
Blastic Natural Killer Cell Lymphoma OR CD4/CD56 Hematodermic Neoplasm
What are the AE of ponatinib?
Arterial and Venous Thrombosis; therefore only approved for CML harboring T315I (either chronic, accelerated, or blast phase) or Ph+ ALL harboring T315I
What TKI is reasonable for Ph+ ALL harboring a T315I mutation?
Ponatinib
What is a major AE of Nilotinib?
QTc prlongation and Pancreatitis
Blastic Plasmacytoid Dendritic Cell Neoplasm can sometimes present with indolent cutaneous involvment known as ______
Hematodermic (this is why it was called CD4/CD56 Hematodermic Neoplasm
What value of RT-PCR for BCR-ABL defines it as Major Molecular Remission?
<0.2 % (so even if increase from 0.05 to 0.1% that is still MMR)
What if there is a cytologic relapse at the 18 month check up after being on TKIs?
Need to switch TKIs and check BCR-ABL mutation status as, depending on the findings, a different TKI may be in order.
T/F: sometimes CML blast crises can have lymphoblastic characteristics
True. If the flow shows TdT+ and other lymphoblastic phenotypic characteristics then it is lymphoblastic blast crisis; Treat like a Ph+ ALL
What if a pt has CML and there are >20% blasts staining positive for TdT?
It can be an ALL blast crisis, this is possible with CML
The DASISION study compared Imatinib vs. Dasatinib as upfront tx for Chronic Phase CML? What was found?
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
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?
They should be on maintenance TKI and you should follow q6 months x3 years
What TKIs are approved first line for chronic phase CML? Which are approved 2nd line?
Imatinib, Dasatinib, and Nilotinib; Ponatinib and Bosutinib
What is a major AE of Dasatinib?
Pleural Effusions
If checking a BCR-ABL mutation status reveals F317L you should use ______
Nilotinib
T/F: the dosing of TKIs differs depending on the phase of CML
True. All TKIs are dosed higher in accelerated phase (AP CML) vs. chronic phase
What are some AE of Imatinib?
Myalgias/Arthralgias and periorbital edema
Is it possible for a pt w/ CML to have cytogenetics not showing t(9;22)?
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
What do you do if someone with CML is failing a TKI?
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
What is the preferred treatment for Blastic Plasmacytoid Dendritic Cell Neoplasm?
An acute leukemia-type regimen followed by allo-HSCT
What TKI in CML should be avoided in ppl with CHF?
Dasatinib
What TKI can cause pleural effusions?
Dasatinib; avoid in CHF
What TKI in CML can cause venous and arterial thromboses?
Ponitinib; only used if T315I BCR-ABL mutation
What is an MMR? CCyR?
Major Molecular Remission; Complete Cytological Remission
What rare hematologic disease, characterized as an acute leukemia is made of a clonal proliferation of precursors of plasmacytoid dendritic cells?
Blastic Plasmacytoid Dendritic Cell Neoplasm
If checking a BCR-ABL mutation status reveals V299L or T315A you should use _____
Nilotinib
What do you do if a pt with CML has MMR and the BCR-ABL transcript increases from 0.05 to 0.1%?
Just keep monitoring because <0.2% is still MMR
If checking a BCR-ABL mutation status reveals Y253H, E255K, or F359V you should use ______
Dasatinib
What is Blastic Plasmacytoid Dendritic Cell Neoplasm?
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
What is the significance of T315I in CML?
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
Can Imatinib be used in pregnancy?
No, it can cause fetal malformations
What CML TKI can cause bad diarrhea and is approved as 2nd line?
Bosutinib
Which TKI is best for CML pts harboring T315I?
Ponatinib; otherwise you hold off on this drug as it causes arterial and venous thromboses
What is the Leukocyte alkaline phosphatase score in CML?
Low; high in leukemoid reactions
