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polycythaemia rubra vera


  • ~10 new cases per million population per year
  • incidences of polycythaemia vera, essential thrombocythaemia, and myelofibrosis were 10x higher among Ashkenazi Jews in northern Israel than in persons of Arabic descent in the region.
  • most cases occur in those 40-60 yrs old and are due to acquired (not inherited) mutations in genes such as JAK2, TET2
  • rarely, risk of PRV may be inherited in an autosomal dominant manner

clinical features

lab tests

  • FBE:
    • raised haematocrit > 0.52 in men and > 0.48 in women
    • raised RBC mass > 25% above normal
    • neutrophis and basophils may be raised
    • raised platelet count in 50% of patients
  • low ESR due to increase in zeta potential
  • JAK2 mutation is strongly associated
    • those who are JAK2 negative may need bone marrow biopsy


  • there is no current curative Rx, but most patients have an almost normal life span with life-long treatment
  • low dose aspirin seems to halve the risk of thrombotic complications in those with no PH thrombotic events 1)
  • asymptomatic patients aged < 40 years can be considered for therapeutic phlebotomies alone to maintain a hematocrit level of less than 45% although this Rx is being re-evaluated
  • other patients can undergo myelosuppressive therapy with hydroxyurea 500 mg PO twice per day (titrated to effect)
    • appears to reduce stroke risk by a further 30%
    • these patients may eventually develop myelofibrosis
    • Ruxolitinib (Jakafi) 10 mg PO twice per day appears to be superior to hydroxyurea for disease control HOWEVER:
      • serious withdrawal symptoms make gradual dose reduction important
      • 82% developed anaemia, 67% thrombocytopenia and 15% neutropenia hence need to monitor FBE
  • radioactive phosphorous can be used as an alternative therapy in older patients
prv.txt · Last modified: 2023/01/09 04:45 by wh

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