Polycythemia Rubra Vera

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Polycythemia rubra vera

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Polycythemia Rubra Vera

  1. 1. Definition:Increase in Hb above normal• Male: 17g/dL• Female: 15g/dLIncrease in HCTMale: > 50%Female: > 45%
  2. 2. Features of the Clinical History• Smoking history• Living in high altitude• Hx of congenital heart disease• Hx of sleep apnea• Hx of COPD
  3. 3. • Incidence: 1.5/100,000• Peak: 45-60 yrs old• Signs:Elevated Hb, WBC, PltMCV normal or microcyticSplenomegaly (60%)
  4. 4. Presentation determined by:Hyperviscosity and thrombosisWBC turnover (gout)Bruising, epistaxis, bleeding from GITNeurologic sx: vertigo, tinnitus, headache,visual disturbances
  5. 5. PE• Ruddy complexion• Splenomegaly• Cyanosis – R-L shunt  CHD• Hypoxemia
  6. 6. Diagnosis- detected incidentally after a routine FBCRBC mass>125%Leukocyte ALP highEPO levels: normal or lowO2 saturation: normal
  7. 7. TreatmentKeep HCT <42% by phlebotomyHydoxyurea: (10-20mg/kg/d PO): used to control WBC/Plt
  8. 8. Prognosis• Often remain well for decades
  9. 9. • Complications:1. Extramedullary hematopoiesis2. Myelofibrosis3. Marrow failure/leukemia
  10. 10. http://crisbertcualteros.page.tl

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