Chronic Leukaemia

Question 1

Define chronic myeloid leukaemia.

Answer 1

Clonal disorder of a pluripotent stem cell in the myeloid lineage of haemopoeisis

Question 2

List 2 causes of CML.

Answer 2

Ionising radiation
Philadelphia chromosome (BCR-ABL fusion protein - continuous JAK stimulation)

Question 3

Describe the clinical features of CML. (6)

Answer 3

Asymptomatic
Fatigue
Weight loss
Night sweats
Abdominal discomfort
Splenomegaly

Question 4

What investigations would you do for CML? (4)

What is the main diagnostic feature? (1)

Answer 4

Blood film
Cytogenetic testing, e.g.
-PCR (for BCR-ABL)
-FISH (for BCR-ABL)
-Karyotyping 

NOTE: if BCR-ABL isn’t present, it’s not CML

Question 5

What are the 3 stages of CML?

Answer 5

Chronic phase
Accelerated phase
Blast/acute phase (blast crisis, blast transformation)

Question 6

Describe the features of the chronic phase in CML. (3)

Answer 6

Blasts: <10%
Asymptomatic
Lasts 3-5 years

Question 7

Describe the features of the accelerated phase in CML. (3)

Answer 7

Blasts: 10-30%
Symptoms begin to increase
Lasts 12-18 months

Question 8

Describe the features of the blast phase in CML. (3)

Answer 8

Blasts: 30+%
Turns into acute leukaemia
Develops over 3-6 months

Question 9

How would you treat CML? (1)

Which drug would you use?

Answer 9

Immunotherapy - imatinib (anti-BCR-ABL)

Question 10

Define chronic lymphoid leukaemia.

Answer 10

Clonal disorder of a pluripotent stem cell in the lymphoid lineage, resulting in abnormal B lymphocytes

Question 11

List 2 causes of CLL.

Answer 11

Idiopathic
Cytogenetics, e.g.
-17p deletions
-11q22 (ATM) mutation

Question 12

Describe the clinical features of CLL.

Answer 12

Asymptomatic
Lethargy
Night sweats
Weight loss
Anaemia
Lymphadenopathy
Infection

Question 13

What investigations would you do for CLL? (4)

Answer 13

Flow cytometry

Peripheral blood film:

• Clonal population of B cells
• CD5, 19, 20 and 23 positive B cells

Bone marrow aspirate:

• Lymphocyte infiltrate (diffuse/nodular)
• Clonal population of B cells

Lymph node biopsy

Question 14

Which staging system would you use for CLL?

Describe how this system works. (3)

Answer 14

Binet staging

A - <3 nodes involved
B - 3+ nodes involved; liver and spleen involved
C - anaemia or thrombocytopenia present

Question 15

List 2 complications of CLL.

Answer 15

Immune complications, e.g.

• Autoimmune haemolytic anaemia
• Autoimmune thrombocytopenia

Infection, e.g.
-Pulmonary infection

Question 16

How would you treat CLL? (3)

Give examples of drugs used for CLL.

Answer 16

Combination chemotherapy:

• R-CHOP (chemo with rituximab)
• Alkylating agents
• Purine analogues

Immunotherapy:

• Rituximab
• Campath-1H (antiCD52+)

Steroids:
-Prednisolone