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Haematology > Chronic Myeloid Leukaemia > Flashcards

Chronic Myeloid Leukaemia Flashcards

1
Q

What is chronic myeloid leukaemia?

A

Leukaemia that is almost exclusively an adult disease, accounting for 14% of all leukaemias. CML affects the myeloid cells, i.e. basophils, neutrophils and eosinophils.

Leukaemic cells retain ability to differentiate, and proliferation occurs without bone marrow failure

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2
Q

What is the natural progression of CML?

A

Chronic phase → aggressive/accelerated phase → blast phase/crisis.

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3
Q

Which age range does CML most commonly occur in?

A

Between 40 and 60 years - disease is almost exclusively in adults

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4
Q

What chromosomal abnormality is CML associated with?

A

Philedelphia chromosome t(9;22)

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5
Q

What is the Phildelphia chromosome?

A

Hybrid chromosome comprising reciprocal translocation between the long arm of chromosome 9 and the long are of chromosome 22, forming a fusion gene BCR/ABL on chromosome 22, which has tyrosine kinase activity

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6
Q

What symptoms can someone with CML present with?

A
  • Tiredness
  • Abdominal discomfort - splenomegaly
  • Weight loss
  • Fever, Night sweats
  • Headache (occasionally) - hyperleucocytosis
  • Priapism - hyperleucocytosis
  • Bruising, bleeding (uncommon)
  • Gout
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7
Q

What signs might you see in someone with CML?

A
  • Pallor - anemia
  • Bruising
  • Splenomegaly - often massive
  • Lymphadenopathy
  • Extramedullary soft tissue leukaemic deposit ‘chloroma’
  • Retinal haemorrhage
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8
Q

What is characteristic of the splenomegaly seen in CML?

A

It is often massive, causing abdominal discomfort

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9
Q

What can ymphdenopathy in CML indicate?

A

Blast crisis

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10
Q

What can retinal haemorrhage indicate in CML?

A

Leucostasis

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11
Q

What investigations might you consider doing in someone with suspected CML?

A
  • Bloods - FBC, U+E’s, LDH, Urate, Blood film, B12
  • Imaging - None
  • Specific - Bone marrow biopsy with cytogenetics, qPCR and FISH
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12
Q

What does a chloroma indicate?

A

Blast crisis

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13
Q

What are features of the chronic phase of CML?

A

Lasting months or years

  • Few if any symptoms
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14
Q

What are features of the accelerated phase of CML?

A
  • Increasing symptoms
  • Increasing spleen size
  • Difficulty in controlling counts
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15
Q

What are features of a blast crisis in CML?

A

Features of acute leukaemia +/- death

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16
Q

What might you see on FBC in someone with CML?

A
  • Normochromic normocytic anaemia
  • WBC raised - usually >100 × 109/L
    • All WBC types of myeloid cell line raised
  • Platelets - low, normal or raised
17
Q

What might you see on blood film in someone with CML?

A
  • Neutrophilia + whole spectrum of mature myeloid precursors.
  • Elevated basophils and eosinophils
  • Increased numbers of blasts
18
Q

What would elevated blast cells on blood film analysis indicate?

A

Accelerated phase/blast crisis

19
Q

What might you see on serum urate in someone with CML?

A

Increased Urate - due to purine breakdown

20
Q

What might you see on B12 blood tests in someone with CML?

A

Increased B12

21
Q

Why would you request FISH/PCR analysis when investigating CML?

A

Used to demonstrate the cytogenetic/molecular abnormality. These are also used to quantitatively monitor response to therapy.

22
Q

How would you manage someone with CML?

A

Tyrosine kinase inhibitors - Direct inhibitors of BCR-ABL fusion :

  • Imatinib (Glivec)
  • Dasatinib (Sprycel)
  • Nilotinib (Tasigna)

Hydroxycarbamide

Allogeneic Transplantation - Only in TKI failures

23
Q

What are side effects of imatinib?

A
  • Nausea
  • Cramps
  • Oedema
  • Rash
  • Headache
  • Arthralgia
24
Q

What are the main chemotherapeutic medications used in CML?

A

Tyrosine kinase inhibitors

25
Q

When is it recommended that stem cell transplantation be used as first line treatment in CML?

A

Young adults

26
Q

How is myeloblastic transformation (blast crisis) treated?

A

Allogeneic stem cell transplantation

Chemotherapy rarely achieves lasting remission

27
Q

What proportion of CML patients have philedelphia chromosome?

A

>80%

28
Q

How would you distinguish between the spleen and a kidney on examination?

A

You can get above the kidney, but not the spleen.

Haematology (34 decks)

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