CHRONIC MYELOID LEUKAEMIA Flashcards Preview

HAEMATOLOGY > CHRONIC MYELOID LEUKAEMIA > Flashcards

Flashcards in CHRONIC MYELOID LEUKAEMIA Deck (26):
1

In chronic myeloid leukaemia, are the leukaemia cells functionless?

No, in fact they almost function normally.

2

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

50-70

3

How are most people with chronic myeloid leukaemia diagnosed?

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

4

What are the known risk factors of chronic myeloid leukaemia?

Radiation history

5

What are the three phases of chronic myeloid leukaemia?

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

6

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

10-20 years

7

What is the Philadelphia chromosome?

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.

8

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

bcr-abl which generates the protein p210Bcr-Abl

9

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

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

10

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

Splenomegaly
Pallor
Tachycardia
Bruising

11

How is the diagnosis of chronic myeloid leukaemia confirmed?

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

12

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

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

13

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

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.

14

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

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

15

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

Imatinib mesylate

16

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

Cryopreservation of semen

17

How does imatinib work in patients with chronic myeloid leukaemia?

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

18

What is the standard dose of imatinib?

400mg/day or more

19

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

>95%

20

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

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

21

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

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

22

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

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

What are the side effects of interferon-alpha?

Common:
Short term fever
Flu-like symptoms

Rare:
Anorexia
Weight loss
Depression
Alopecia
Rashes
Neuropathies
Autoimmune
Thrombocytopenia

24

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

Excessive sweating
Persistent fever
Unexplained anaemia
Splenomegaly
Bleeding
Bone pain

25

What is a chloroma?

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

How is someone with advanced phase chronic myeloid leukaemia managed?

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