Polycythemia rubra vera

Question 1

Polyxythemia rubra vera

Answer 1

increase in red cells with no apparent cause

Question 2

Diagnosis

Answer 2

●Increased hemoglobin level or other evidence of increased red cell volume
●Bone marrow biopsy showing hypercellularity
●JAK2 mutation

●Serum erythropoietin level below the reference range for normal

Question 3

Jak 2 mutation

Answer 3

tyrosine kinase inhibitor
leads to epo independent proliferation
found in 90% of polycythemia rubra vera

Question 4

secondary causes of increased hemocrit

Answer 4

dehydration

hypoxia:

smoking
sleep apnea
high altitude

Question 5

treatment of polycythemia vera

Answer 5

decrease red cell mass

phlebotomy
aspirin

hydroxyurea / chemotherapy rarely required

Question 6

symptoms of polycythemia vera

Answer 6

headache
visual disturbances
occlusive disease
splenomegaly