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HAEMATOLOGY > CHRONIC MYELOID LEUKAEMIA > Flashcards

CHRONIC MYELOID LEUKAEMIA Flashcards

1
Q

In chronic myeloid leukaemia, are the leukaemia cells functionless?

A

No, in fact they almost function normally.

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2
Q

What is the peak age of incidence of chronic myeloid leukaemia?

A

50-70

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3
Q

How are most people with chronic myeloid leukaemia diagnosed?

A

From routine blood tests - they normally do not show any symptoms at the time of diagnosis

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4
Q

What are the known risk factors of chronic myeloid leukaemia?

A

Radiation history

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5
Q

What are the three phases of chronic myeloid leukaemia?

A

Chronic or stable phase
Accelerated phase
Advanced phase (acute and blastic)

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6
Q

With modern treatment, how long does the chronic phase of chronic myeloid leukaemia last on average?

A

10-20 years

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7
Q

What is the Philadelphia chromosome?

A

Also known as 22q-. This is where there a t(9;22) translocation so that chromosome 22 is shorter than its unaffected counterpart and chromosome 9 is longer than its unaffected counterpart. It is diagnostic of chronic myeloid leukaemia.

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8
Q

What is the name of the specific fusion gene on the Philadelphia chromosome which is responsible for chronic myeloid leukaemia?

A

bcr-abl which generates the protein p210Bcr-Abl

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9
Q

How do patients with symptoms of chronic myeloid leukaemia classically present?

A 
Fatigue
Weight loss
Sweating
Anaemia
Haemorrhage
Sensation of a mass in the left upper abdominal quadrant
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10
Q

On examination, what might you find in someone with chronic myeloid leukaemia?

A

Splenomegaly
Pallor
Tachycardia
Bruising

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11
Q

How is the diagnosis of chronic myeloid leukaemia confirmed?

A

Blood tests
Blood film
Bone marrow
Reverse transcriptase to confirm Bcr-Abl

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12
Q

What would the blood film of someone with chronic myeloid leukaemia show?

A

Greatly increased number of leucocytes with many immature forms (promyelocytes and myelocytes)

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13
Q

What would the bone marrow of someone with chronic myeloid leukaemia show?

A

Increased cellularity with immature leucocytes as seen on blood film.
Red cell production is reduced.
Megakaryocytes are plentiful but may be smaller than usual and look slightly atypical.

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14
Q

What would blood tests of someone with chronic myeloid leukaemia show?

A 
Raised white cell count
Increased basophils and eosinophils
Low Hb
Often raised platelet count
Raised lactate dehydrogenase
Raised urate
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15
Q

What is the drug of choice for someone diagnosed with chronic myeloid leukaemia who is found to still be in the chronic phase?

A

Imatinib mesylate

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16
Q

As well as imatinib, what should all young men with chronic myeloid leukaemia be offered?

A

Cryopreservation of semen

17
Q

How does imatinib work in patients with chronic myeloid leukaemia?

A

Inhibits enhanced protein kinase activity of Bcr-Abl oncoprotein and hence induces apoptosis in such cells

18
Q

What is the standard dose of imatinib?

A

400mg/day or more

19
Q

What is the success of rate of complete haematological remission in chronic myeloid leukaemia patients taking imatinib?

A

> 95%

20
Q

What is the best way to monitor the patient’s response to imatinib, the treatment for chronic myeloid leukaemia?

A

Regular assay if Bcr-Abl transcript numbers in peripheral blood using real time quantitative RT-PCR technology.

21
Q

In patients who cannot tolerate or do not respond to imatinib, what are the alternatives for treatment of chronic phase chronic myeloid leukaemia?

A

Allogeneic stem cell transplantation from a HLA identical sibling
Second generation tyrosine kinase inhibitor such as Dasatinib or Nilotinib
Hydroxyurea
Interferon-alpha

22
Q

When do you prescribe hydroxyurea for a patient with chronic phase chronic myeloid leukaemia?

A

It is a useful drug to control symptoms in the short-term post diagnosis or as an interim measure for those who are resistant to imatinib.

23
Q

What are the side effects of interferon-alpha?

A

Common:
Short term fever
Flu-like symptoms

Rare:
Anorexia
Weight loss
Depression
Alopecia
Rashes
Neuropathies
Autoimmune
Thrombocytopenia
24
Q

How does someone with more advanced phase chronic myeloid leukaemia present?

A 
Excessive sweating
Persistent fever
Unexplained anaemia
Splenomegaly
Bleeding
Bone pain
25
Q

What is a chloroma?

A

A solid focus of leukaemia outside of the bone marrow. Seen during a blast crisis - when further damage to DNA means that it has now added a proliferative effect and so starts proliferating quickly.

26
Q

How is someone with advanced phase chronic myeloid leukaemia managed?

A

Short-term benefit from imatinib - might re-establish chronic phase - if diagnosed for the first time (not useful in patients who have progressed despite being on imtinib)

Hydroxyurea

Splenomectomy can improve symptoms

Appropriate chemotherapy - treated as if AML or ALL

Prophylaxis against neuroleukaemia with methotrexate

HAEMATOLOGY (17 decks)

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