Polycythemia Flashcards
What is polycythemia?
Excessive production in red cells
What is seen in an fbc in polycythemia? (2)
Raised Hb conc
Raised haematocrit
What is polycythemia Vera?
Primary polycythemia
What is the genetic mutation that causes polycythemia Vera?
JAK2 (Janus Kinase 2) gene
What are some causes of secondary polycythemia? (3)
Chronic hypoxia e.g. smoking / COPD
Local renal hypoxia e.g. renal artery stenosis
Excess EPO production from EPO secreting tumours
What are 2 risk factors for polycythemia? (2)
60-70yo
History of budd-chiari syndrome
What are some clinical signs of polycythemia? (4)
• A ‘ruddy’ (reddish) complexion
• Splenomegaly: present in one-third of patients at the time of diagnosis
• Abdominal masses: benign and malignant uterine, renal and hepatic tumours which can secrete EPO may be palpable
• Hypertension
What are the Hb and haematocrit ranges for polycythemia in men and women?
○ Haemoglobin (Hb) >185 g/L and/or haematocrit (Hct) > 0.52 in males
○ Hb >165 g/L and/or Hct > 0.48 in females
What is relative polycythemia?
• If the patient is dehydrated, apparent polycythaemia may be present in which the Hb/Hct is raised because of a reduced plasma volume. These patients will have a normal red cell mass. = relative polycythaemia
What does polycythemia increase a persons risk of?
Increased risk of arterial and venous thrombosis
What is the management of polycythemia? (3)
Phlebotomy - intermittent long-term venesection
Aspirin - low dose to reduce risk of thrombotic events
Cytoreductive therapy e.g. hydroxycarbamide
What are the complications of polycythemia? (6)
• Ischaemic stroke
• Myocardial infarction
• Pulmonary embolism
• Progression to myelofibrosis or acute myeloid leukaemia
• Gastrointestinal haemorrhage
• Budd-Chiari syndrome
What is the prognosis of polycythemia?
14 Years
What is the most common cause of death in polycythemia?
Thrombolytic events
