Chronic Myeloid Leukemia

Question 1

CML is driven by ___ that codes for a constitutively active tyrosine kinase, resulting from a reciprocal balanced translocation between long arms of chromosomes 9 and 22 t(9;22), known as the ___?

Answer 1

BCR-ABL1 chimeric gene; Philadelphia chromosome

Question 2

True or false. The course of CML is typically biphasic or triphasic, with an early indolent or chronic phase, followed often by an accelerated phase and a terminal blastic phase.

Answer 2

True

Question 3

True or false. In untreated CML, leukocytosis ranging from 10-500x10^9/L is common.

Answer 3

True

Question 4

True or false. In CML, thrombocytosis is common, but thrombocytopenia is rare and, when present, suggests a worse prognosis.

Answer 4

True

Question 5

In CML, the bone marrow is hypercellular with marked myeloid hyperplasia and a high myeloid-to-erythroid ratio of __?

Answer 5

15-20:1

Question 6

In CML, marrow blasts are 5% or less; when higher, they carry a worse prognosis or represent transformation to accelerated phase (if they are >___%)

Answer 6

15

Question 7

Monitoring patients on TKI therapy by cytogenetics, FISH, and molecular studies has become an important standard practice to assess response to therapy, emphasize compliance, evaluate possible treatment resistamce, identify the need to change TKI therapt, and detemine the need to assess for kinase domain mutations. A partial cytogenetic response is defined as the presence of ___ or less Ph-positive metaphases by routine cytogenetic analysis.

Answer 7

35% or less

Question 8

Which is true of accelerated-phase CML?
A. Presence of 15% or more of peripheral blasts
B. 30% or more peripheral blasts plus promyelocytes
C. 20% or more peripheral basophils
D. Cytogenetic clonal evolution (presence of chromosomal abnormalities in addition to Ph)
E. Thrombocytopenis <100x10^9/L (unrelated to therapy)

Answer 8

All are true

Question 9

Blastic-phase CML is defined by the presence of ___ peripheral or marrow blasts or the presence of sheets of blasts in extramedullary disease.

Answer 9

30% or more

Question 10

With TKI therapy, the estimated 10-year survival in CML is __.

Answer 10

85%

Question 11

True or false. A general practice rule is to continue the particular TKI chosen at the most tolerable dose schedule jot associated with grade 3-4 side effects or with bothersome chronic side effects, for as long as possible, until either with cytogenetic relapse or the persistence of unacceptable side effects.

Answer 11

True

Question 12

These two factors are the indicators of failure of a particular TKI therapy.

Answer 12

1. Cytogenetic relapse

2. Intolerable side effects as judged by the patient and treating physician

Question 13

Side effects of TKI

Answer 13

> Imatinib- renal

> Nilotinib, dasatinib, ponatinib- arterio-occlussive disease

Question 14

Which TKI may produce QTc prolongation?

Answer 14

Nilotinib and dasatinib