Polycythaemia

Question 1

Define polycythaemia rubra vera

Answer 1

Clonal haematological malignancy with risk of thrombosis and potential to evolve to myelofibrosis and AML

Question 2

Ix polycythaemia rubra vera?

Answer 2

• JAK2 mutation
• RBCS, WCCs, platelets, HCT, PCV, B12 up
• Bone marrow - hyperplasia
• Cytogenetics to differentiate from CML
• Serum EPO down

Question 3

Sx polycythaemia rubra vera?

Answer 3

• Pruritus after bath
• Headaches, tinnitus, dizzines
• Erythromelalgia (burning in digits)
• Facial plethora
• Splenomegaly
• Gout
Arterial or venous thrombosis history

Question 4

Mx polycythaemia rubra vera

Answer 4

• Aim to lower risk of thrombosis
• Aim to keep HCT <0.45
• Venesection in younger lower risk patients
• Hydroxycarbamide in older patients
Aspirin 75mg OD

Question 5

secondary causes of polycythaemia?

Answer 5

COPD

High altitude

Cyanotic heart disease

Heavy smoking

EPO-producing tumour (renal and hepatic)