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prv

polycythaemia rubra vera

see also:

introduction

  • ~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

treatment

  • Rx is palliative
  • asymptomatic patients aged < 40 years can be considered for therapeutic phlebotomies alone to maintain a hematocrit level of less than 45%
  • other patients can undergo myelosuppressive therapy with hydroxyurea
  • radioactive phosphorous can be used as an alternative therapy in older patients
prv.txt · Last modified: 2014/11/24 21:12 (external edit)