Polycythemia Vera

Question 1

What is Polycythemia Vera?

Answer 1

myleoproliferative disorder, clonal proliferation of marrow stem cell = increase in red cell volume

Question 2

what other blood cell types are often overproduced in Polycythemia Vera?

Answer 2

Neutrophilia and thrombocythemia

Question 3

What mutation is associated with Polycythemia Vera?

Answer 3

JAK2 inhibitor

Question 4

What age develop Polycythemia Vera?

Answer 4

60-70 yrs

Question 5

Presentation of Polycythemia Vera?

Answer 5

• pruritus, typically after a hot bath
• splenomegaly
• hypertension
• hyperviscosity
  
  • arterial thrombosis
  • venous thrombosis
• haemorrhage (secondary to abnormal platelet function)

Question 6

Investigation of Polycythemia Vera?

Answer 6

FBCs (raised raised haematocrit; neutrophils, basophils, platelets raised in half of patients)
JAK2 mutation
serum ferritin
U&Es; LFTs

Question 7

Laboratory findings of polycythemia vera?

Answer 7

Raised:
- Hb, Hct, RBC, total red cell volume
leucocytes, neutrophils, basophils
platelets
in 95% patients: JAK2 mutation, peripheral blood granulocytes
plasma urate, LDH

Decreased:
- EPO
-ESR

Question 8

Mangement of polycthemia vera?

Answer 8

• aspirin
  
  • reduces the risk of thrombotic events
• venesection = removes 1 pint (half a litre) of blood at a time
  
  • first-line treatment to keep the haemoglobin in the normal range
• chemotherapy
  
  • hydroxyurea (hydroxycarbamide) - slight increased risk of secondary leukaemia
  • phosphorus-32 therapy

Question 9

Prognosis of of polycythemia vera?

Answer 9

can lead to myelofibrosis (too few blood cells)
can lead to acute leukaemia (increased risk with chemo treatment)