Myeloproliferative disorder, increase red cell count, can be assoc with increased WCC and platelets too. Underlying aetiology = JAK2 mutation allowing the tyrosine kinase to be hypersensitive to EPO. Epo levels are usually decreased. Symptoms = pruritis especially when limb put in warm water. Erythromelalgia = pain + discolouration of a limb. Gouty arthritis and increased risk of thrombotic events inc budd-chiari.
- Diagnose with genetics for JAK2 mutation.
- Red cell mass + haematocrit secondline.
Treat with phlebotomy, low dose aspirin, hydroxyureacarbamide to decrease RBC prolif.