Chronic Myeloproliferative Disorders

Question 1

What is the pathophysiology behind myeloproliferative disorders?

Answer 1

Abnormal proliferation of myeloid cells in the bone marrow

Question 2

Which chronic myeloproliferative disorder is BCR-ABL1 positive?

Answer 2

Chronic myeloid leukaemia

Question 3

What are some signs and symptoms which are common to all myeloproliferative disorders?

Answer 3

Symptoms of increased cell turnover, splenomegaly, arterial/venous thrombosis

Question 4

What are some symptoms of increased cell turnover which may be seen in patients with myeloproliferative disorders?

Answer 4

Fatigue, weight loss, night sweats, gout

Question 5

An increase in the number of which cells is highly suggestive of a myeloproliferative disorder rather than a reactive cause?

Answer 5

Eosinophils and basophils

Question 6

When does the incidence of polycythaemia vera peak?

Answer 6

6th decade of life

Question 7

What is the pathophysiology behind polycythaemia vera?

Answer 7

A high haemoglobin/haematocrit accompanied by erythrocytosis

Question 8

What are the two main secondary causes of polycythaemia?

Answer 8

Chronic hypoxia or inappropriately high Epo production

Question 9

What are some potential causes of pseudo-polycythaemia?

Answer 9

Dehydration, diuretic use, obesity

Question 10

What are some symptoms that may be experienced by individuals with polycythaemia vera due to hyperviscosity of blood?

Answer 10

Headache and visual disturbances

Question 11

An itch which is worsened on exposure to hot water is most suggestive of what diagnosis?

Answer 11

Polycythaemia vera

Question 12

95% of patients with polycythaemia vera have a mutation in what protein?

Answer 12

JAK2

Question 13

What is the first line investigation for polycythaemia vera?

Answer 13

JAK2 mutation status

Question 14

When treating polycythaemia vera, the aim is for a haematocrit level of less than what?

Answer 14

0.45

Question 15

What treatment is used to decrease the haematocrit in patients with polycythaemia vera who are young and low-risk?

Answer 15

Venesection

Question 16

What treatment is used to decrease the haematocrit in patients with polycythaemia who are high-risk, i.e. aged > 60 or previous thrombotic event?

Answer 16

Hydroxyurea

Question 17

When treating myeloproliferative disorders, what drug is preferred over the use of hydroxyurea in young women of child bearing age?

Answer 17

Alpha interferon

Question 18

What treatment is given to patients with polycythaemia vera and essential thrombocytosis to reduce the risk of thrombosis?

Answer 18

Low dose aspirin

Question 19

How often should FBC be monitored in patients with polycythaemia vera?

Answer 19

Every 3 months

Question 20

The risk of both thrombosis and haemorrhage is increased in patients with which myeloproliferative disorder?

Answer 20

Essential thrombocytosis

Question 21

A burning sensation in the hands is a characteristic feature of which myeloproliferative disorder?

Answer 21

Essential thrombocytosis

Question 22

What mutation may be present in patients with essential thrombocytosis who are negative for JAK2?

Answer 22

CALR

Question 23

What is the first line treatment for essential thrombocytosis?

Answer 23

Hydroxyurea

Question 24

Myelofibrosis can be idiopathic or can develop as a result of what other conditions?

Answer 24

Polycythaemia vera or essential thrombocytosis

Question 25

A blood film showing tear-drop shaped red blood cells (poikilocytes) is suggestive of what diagnosis?

Answer 25

Myelofibrosis

Question 26

How is myelofibrosis diagnosed?

Answer 26

Bone marrow biopsy

Question 27

What treatment may be curative in young patients with myelofibrosis but comes with a high risk of mortality?

Answer 27

Allogenic stem cell transplant

Question 28

Ruxolitib is an example of what type of drug?

Answer 28

JAK2 inhibitor

Question 29

What is the mean survival after diagnosis of myelofibrosis?

Answer 29

4-5 years