Polycythaemia Flashcards
1
Q
What is polycythaemia rubra vera?
A
- Also known as primary polycythaemia
- Myeloproliferative disorder
- Clonal expansion of stem cells leading to increased red cell volume
- Often associated with increased neutrophil and platelet production
-95% associated with JAK2 MUTATION
2
Q
What are secondary causes of polycythaemia?
A
- COPD
- altitude
- obstructive sleep apnoea
- excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids
3
Q
What is the aetiology of PRV?
A
- Peak incidence in 60s
- Male > female
4
Q
What are the symptoms of PRV?
A
- Headches
- Dizziness
- Pruritus after bathing
- Loss of consciousness
- Gout
5
Q
What are the investigation for PRV?
A
BLOODS/FILMS
- FBC: increase haematocrit, neutrophils, basophils, platelets
- JAK2 mutation
- serum ferritin
- renal and liver function tests
6
Q
What additional tests can be done if JAK2 negative but PRV highly suspected?
A
red cell mass arterial oxygen saturation abdominal ultrasound serum erythropoietin level bone marrow aspirate and trephine cytogenetic analysis erythroid burst-forming unit (BFU-E) culture
7
Q
How is PRV managed?
A
- aspirin
- venesection - first line treatment
- hydroxyurea -slight increased risk of secondary leukaemia
- phosphorus-32 therapy
