Haem - CML

Question 1

CML - sex? age?

Answer 1

Men>F

40-60 years

Question 2

Typical CML history?

Answer 2

Lethargy/hypermetabolic state (SOB, hyperuricemia, wt loss)/THROMBOTIC EVENT E.G. MONOCULAR BLINDNESS

Question 3

Typical finding on examination of CML patient? why?

Answer 3

massive splenomegaly - chronic therefore time for cells to accumulate

Question 4

FBC result on CML

Answer 4

MASSIVE leukocytosis >50 x10^9

Question 5

Blood film results in CML?

Answer 5

Raised basophils
Raised neutrophil and promyelocytes
<5% BLASTS (if more –> acute leukaemia :( )

Question 6

Natural, untreated history of CML

• what are the 3 phases?

Answer 6

1. Chronic
2. Accelerated
3. Blast crisis

Question 7

In what disease phase are 80% of CML patients diagnosed?

Answer 7

Chronic phase

Question 8

What is the difference between chronic phase and accelerated phase?

Answer 8

Chronic: <5% of cells are blasts
Accelerated: 10-19% are blasts

Question 9

Key mutation seen in CML?

Answer 9

Philadelphia chromosome
t(9;22)
This expresses a fusion gene with TK activity

Question 10

Most sensitive away to identify leukaemia cells in the body?

Answer 10

RT-qPCR

Question 11

3 ways to detect leukaemic cells in the body?

Answer 11

FBC - leukocyte levels
Cytogenetic analysis (FISH)
Molecular analysis (RT-qPCR)

Question 12

3 ways to monitor response to Tx in CML?

Answer 12

FBC
FISH
qPCR

Question 13

Main drug used for CML treatment? MOA?

Answer 13

Imatinib

a TKI

Question 14

Survival rate of CML with imatinib treatment?

Answer 14

95% survival at 5 years

Question 15

Why does imatinib not always work? give 3 reasons

Answer 15

Patient non-compliance

• resistance due to point mutation
• SEs - pleural effusion from fluid retention