Polycythaemia Rubra Vera (PRV)

Question 1

What is Polycythaemia Rubra Vera?

Answer 1

When there are too many red blood cells produced in the bone marrow, due to UNIDENTIFIED CAUSE.

Question 2

What mutation is associated with PRV?

Answer 2

JAK2 Mutation

associated with 95% of people with polycythaemia rubra vera

Question 3

How is polycythaemia rubra vera usually discovered?

Answer 3

Usually asymptomatic,

so often discovered during a routine blood test or physical examination.

Question 4

What are symptoms associated with PRV due to the thickening of the blood? (2)

Answer 4

Intermittent Claudication

Thrombosis / TIA / Strokes

Question 5

What sign can be discovered with an abdominal examination that suggests polycythaemia rubra vera?

Answer 5

Splenomegaly

Question 6

What symptoms may the patient present with if they have polycythaemia rubra vera? (2)

Answer 6

Itchiness

Headache

Question 7

What is the definitive lab result that separates PRV from anaemia? (2)

Answer 7

RAISED

Haemocrit & Haemoglobin

Question 8

What Lab investigations will you look at if a patient is suspected of PRV? (6)

Answer 8

• Hb & Haemocrit
• WBC & platelets
• Uric acid
• Raised RBC mass (when volume is measured)
• Blood film

Question 9

What do you expect to see in a blood film of polycythaemia rubra vera patient?

Answer 9

Microcytosis

• Large and abnormal platelets present

Question 10

What is the treatment for polycythaemia rubra vera? (2)

Answer 10

Venesection
Aspirin

Hydroxycarbamide (some cases)

Question 11

What is the ideal haemocrit level for men and women with PRV?

Answer 11

Men - Below 0.45

Women - Below 0.45