Chronic Myeloid Leukaemia (CML)

Question 1

Leukocyte relates to immune cells of the body. This can be divided into granulocytes (neutrophils, eosinophils, and basophils), monocytes, and lymphocytes (B, T and NK cells). Chronic myeloid leukaemia (CML) is a malignancy of precursor cells from the myeloid lineage. What is the incidence of CML?

1 - 1 cases per 100,000
2 - 10 cases per 100,000
3 - 100 cases per 100,000
4 - 1000 cases per 100,000

Answer 1

1 - 1 cases per 100,000

Slightly more common in males 1.5:1 ratio

Question 2

Leukocyte relates to immune cells of the body. This can be divided into granulocytes (neutrophils, eosinophils, and basophils), monocytes, and lymphocytes (B, T and NK cells). Chronic myeloid leukaemia (CML is a malignancy of precursor cells from the myeloid lineage. What age does the incidence of CML typically peak?

1 - <12 months
2 - 10-20 years
3 - 20-40 years
4 - 60-70 years

Answer 2

4 - 60-70 years

Question 3

When comparing leakaemia and lymphoma, which is a solid and which is a liquid tumour?

Answer 3

• leukaemia = liquid (blood)
• lymphoma = solid (lymph nodes)

Question 4

When comparing leukaemia and lymphoma, which cells are generally affected?

Answer 4

• leukaemia = myeloid and lymphoid cells (all bone marrow cells)
• lymphoma = lymphoid cells (B, T and NK cells)

Question 5

When looking at cell lines from the bone marrow we talk about haemopoietic stem cells, which are then able to differentiate into myeloid and lymphoid stem cells. Which of the following is NOT classed as a myeloid stem cell?

1 - erythrocyte
2 - thrombocyte (platelet)
3 - monocyte
4 - natural killer cell
5 - granulocyte

Answer 5

4 - natural killer cell
- these are classed as lymphoblasts

• granulocyte = eosinophil, neutrophil, basophil

Question 6

When looking at cell lines from the bone marrow we talk about haemopoietic stem cells, which are then able to differentiate into myeloid and lymphoid stem cells. Which of the following is NOT classed as a lymphoblast stem cell?

1 - pre-B cell
2 - thrombocyte (platelet)
3 - Pre-T cell
4 - natural killer cell

Answer 6

2 - thrombocyte (platelet)

Question 7

In chronic myeloid leukaemia (CML) which of the following do the myeloid cells do?

• cells divide too quickly
• cells do not die when they should

Answer 7

• cells divide too quickly

Question 8

One of the most common causes of chronic myeloid leukaemia (CML) is chromosomal abnormalities. Which of the following is NOT a type of chromosomal abnormalities that can lead to CML?

1 - chromosomal deletion
2 - trisomy
3 - isochromosome
4 - translocation

Answer 8

3 - isochromosome

Question 9

Chromosomal abnormalities is one of the most common causes of chronic leukaemia. Which chromosomal abnormality accounts for 80% of all cases of chronic myeloid leukaemia (CML)?

1 - Cri du chat syndrome
2 - Klinefelter syndrome
3 - Turner syndrome
4 - Philadelphia chromosome

Answer 9

4 - Philadelphia chromosome

• 9 and 22 chromosome translocation
• forms a BCR-ABL gene involved in tyrosine kinase which essentially causes rapid uncontrolled cell replication

Genetic testing that confirms this is typically diagnostic of CML

Question 10

Which of the following is NOT true for chronic myeloid leukaemia (CML)?

1 - commonly caused by Philadelphia chromosome
2 - can affect all myeloid cell lineage
3 - rapid onset and progression
4 - basophils are very high in blood, when typically they are low

Answer 10

3 - rapid onset and progression

• typically this is slow onset, but does have some accelerated phases

Question 11

Which environmental risk factor is most commonly associated with causing chronic myeloid leukaemia (CML)?

1 - diet
2 - pollution and toxin
3 - alcohol and smoking
4 - ionising radiation exposure

Answer 11

4 - ionising radiation exposure

Smoking, alcohol, diet, obesity etc.. have also shown some association with CML

Question 12

Patients typically present asymptomatic until the chronic phase, where they can then present with all of the following, EXCEPT which one?

1 - Fatigue/lethargy
2 - Night sweats
3 - Weight gain
4 - all of the above

Answer 12

3 - Weight gain

Typically causes weight loss

Question 13

Once the excessive division of myeloid cells leave the bone marrow they can enter organs and enlarge them. Which 2 organs are most commonly enlarged?

1 - lungs
2 - kidneys
3 - spleen
4 - liver

Answer 13

3 - spleen
4 - liver

Splenomegaly can cause bloating, early satiety, increased abdominal girth, nausea, vomiting.

Question 14

Chronic myeloid leukaemia (CML) can affect all myeloid cell lineages, but which cell is typically elevated in the blood, when normally the levels are low?

1 - basophils
2 - eosinophils
3 - natural killer cell
4 - monocyte

Answer 14

1 - basophils

Question 15

Which of the following is NOT a common sign or symptoms of Chronic myeloid leukaemia (CML)?

1 - hepatosplenomegaly
2 - anaemia and fatigue
3 - bleeding
4 - features of gout
5 - enlarged and tender rubbery lymph nodes

Answer 15

5 - enlarged and tender rubbery lymph nodes

• common in chronic lymphocytic leukaemia (CLL)

Question 16

In chronic myeloid leukaemia (CML) which of the following test results is commonly observed?

1 - high WBC
2 - low Hb
3 - high urate
4 - high B12
5 - all of the above

Answer 16

5 - all of the above

Question 17

Which of the following is NOT a symptom of anaemia in chronic myeloid leukaemia?

1 - Dizziness and shortness of breath
2 - palpitations, pre-syncope and syncope in severe cases
3 - pallor and koilonychia
4 - bradycardia
5 - relative hypotension
6 - signs of high output heart failure such as a gallop rhythm and peripheral oedema

Answer 17

4 - bradycardia

Typically causes tachycardia

Question 18

A patient with suspected chronic myeloid leukaemia (CML) presents to the GP surgery. What is your course of action?

1 - blood tests and meet again in GP surgery in 2 weeks
2 - urgent 2 week referral
3 - standard haematology pathway referral
4 - antibiotics and corticosteroids prescription and review in 2 weeks

Answer 18

2 - urgent 2 week referral

Question 19

If you suspect a diagnosis of chronic myeloid leukaemia (CML) a FBC should be performed. Would we expect to see a micro, normo or macrocytic anaemia?

Answer 19

• normocytic anaemia

Folate, B12 and iron are all present so myeloid cells should be normal size

Commonly see normocytic, normochromic anaemia

Question 20

If you suspect a diagnosis of chronic myeloid leukaemia (CML) a FBC should be performed. Would we expect to see normal, hypo or hyperchromic RBCs?

Answer 20

• normochromic

Commonly see normocytic, normochromic anaemia

Question 21

If you suspect a diagnosis of chronic myeloid leukaemia (CML) a FBC should be performed. Would we expect to see leukopenia or leukocytosis?

Answer 21

• leukocytosis

Myeloid cells differentiate uncontrollably

Question 22

Typically on a blood film, which of the following may be present in chronic myeloid leukaemia (CML)?

1 - leucocytosis and leucoerythroblastic
2 - thrombocytopneia and leucocytosis
3 - leucocytosis and lymphoblastosis
4 - all of the above

Answer 22

1 - leucocytosis and leucoerythroblastic

Leucoerythroblastic = presence of immature RBC and WBC in peripheral blood

May also see:
- accelerated phase = >15% of the blood film are blast cells
- blastic phase = >20% of the blood film is occupied by blastic cells.

Question 23

Will lactate dehydrogenase be high or low in chronic myeloid leukaemia (CML)?

Answer 23

• high

Due to cell lysis and high turnover

Question 24

Following a bone marrow aspirate, how are the findings of the bone marrow aspirate typically described?

1 - hypocellular
2 - hypercellular
3 - normocellular

Answer 24

2 - hypercellular

Question 25

There are 3 phases of chronic myeloid leukaemia (CML). Match the bone marrow aspirate findings with the stage according to the WHO:

• chronic, accelerated and blastic phase
• <10% blasts, <10-19% blasts and >20% blasts in peripheral blood

Answer 25

• chronic = <10% blasts,
• accelerated = <10-19% blasts
• blastic phase = >20% blasts

Question 26

There are 3 stages of chronic myeloid leukaemia (CML). Which of the following is typically fatal?

1 - blastic phases
2 - chronic phase
3 - accelerated phase

Answer 26

1 - blastic phases

Question 27

What is the median survival rate of chronic myeloid leukaemia (CML)?

1 - 1-2 years
2 - 5-6 years
3 - 10-20 years
4 - >35 years

Answer 27

2 - 5-6 years

Question 28

In patients with chronic myeloid leukaemia (CML), what is the definition of Haematologic remission?

1 - <20% blasts in peripheral blood
2 - <15% blasts in peripheral blood
3 - <10% blasts in peripheral blood
4 - no blasts in peripheral blood

Answer 28

4 - no blasts in peripheral blood

No sign of splenomegaly is also important

Question 29

In patients with chronic myeloid leukaemia (CML), what is the definition of cytogenetic remission?

1 - <20% Philadelphia chromosome-positive (Ph+) cells on chromosomal analysis
2 - <10% Philadelphia chromosome-positive (Ph+) cells on chromosomal analysis
3 - No Philadelphia chromosome-positive (Ph+) cells on chromosomal analysis

Answer 29

3 - No Philadelphia chromosome-positive (Ph+) cells on chromosomal analysis

Molecular remission defined as negative polymerase chain reaction result for the mutational BCR/ABL mRNA

Question 30

Which of the following drugs that works by inhibiting tyrosine kinase pathways is first line and is described as the silver bullet in chronic myeloid leukaemia?

1 - methotrexate
2 - imatinib
3 - hydroxyurea
3 - busulfan

Answer 30

2 - imatinib

Patients can often have normal lifespan with this.

Question 31

In chronic myeloid leukaemia (CML) a specific medication has been developed over the last 10 years. What medication is this targeted against?

1 - ensure disconjunction in high risk patients
2 - BCR-ABL inhibitors
3 - all-trans retinoic acid
4 - steroids targeting blast cells

Answer 31

2 - BCR-ABL inhibitors
- accounts for 80% of all CML

This is imatinib

Question 32

Imatinib works by inhibiting tyrosine kinase pathways and is first line drug, described as the silver bullet in chronic myeloid leukaemia (CML). Is Imatinib as effective when started in all 3 phases of CML? (chronic, accelerated and blastic phases)?

Answer 32

• no

Need careful assessment of best time to start the medication

Those in the blastic phase typically have a poor prognosis

Question 33

In patients, where induction therapy has not been successful, they may be offered allogeneic transplantations, which involves a donor, typically a sibling or unrelated donor) providing a sample to help the treat the patient. However, not all patients with chronic myeloid leukaemia (CML) are offered this, and it is rarely a 1st line therapy, why?

1 - doesn’t always work
2 - not enough donors
3 - too expensive
4 - high morbidity and mortality

Answer 33

4 - high morbidity and mortality

Question 34

Are all patients with chronic myeloid leukaemia given haematopoietic stem cell transplantation?

Answer 34

• No

Typically younger patients