Polycythaemia Flashcards
What is polycythaemia?
A Philadelphia chromosome negative myeloproliferative disorder
What is primary polycythaemia?
ruba vera
What is secondary polycythaemia?
- Appropriate (hypoxic response)
- Inappropriate (EPO-secreting tumour
What are RF for polycythaemia?
- Older (around 60)
- Budd-chiari syndrome
What are symptoms for polycythaemia?
- Asymptomatic
- Aquagenic prutirtis
- Hyper viscosity syndrome vague: headache, dizziness
What are signs of polycythaemia?
- Facial plethora
- Splenomegaly
- Gout
How do you distinguish polycythaemia from CML?
cytogenetics
What are differential diagnosis of polycythaemia?
- Secondary polycythaemis owing to hypoxia
- Essential thrombocythemia
- CML
- Polycythaemia due to elevated erythropoietin level
- Congenital polycythaemia
What investigations and results are shown in polycythaemia?
- Elevated Hb, haematocrit, WBC
- elevated platelet count
- +/- thrombocytosis
- low serum erythropoietin
- JAK2 gene mutation screen
Is there a mutation in polycythaemia?
Almost all PCV is JAK2 V617F +ve
What is the management plan for polycythaemia?
- Aim to keep HCT<0.45
- Decrease risk of thrombosis
- Preg or low risk 1st line: Phlebotomy
- Non preg, high risk 1st line: cytoreductive therapy
- Aspirin 75mg daily
- If high risk: Alpha interferon in child bearing and hydroxycarbamide
What are complications of polycythaemia?
- Acute/therapy related Leukaemia
- Thrombosis
- Pruritis
What is prognosis of polycythaemia?
- Transition to myelofibrosis happen 30%
2. Monitor fbc every 3months
What is anti-phospholipid syndrome?
Auto-immune mediated thrombosis – often manifesting during pregnancy
What are the clinical features of antiphospholipid syndrome?
- Recurrent miscarriages (3+)
- VTE
- Stroke/MIs, HTN (arterial problems)
- Livedo reticularis (mottled)
