Polycythemia rubra vera

2. Definition:
Increase in Hb above normal
• Male: 17g/dL
• Female: 15g/dL
Increase in HCT
Male: > 50%
Female: > 45%

3. Features of the Clinical History
• Smoking history
• Living in high altitude
• Hx of congenital heart disease
• Hx of sleep apnea
• Hx of COPD

4. • Incidence: 1.5/100,000
• Peak: 45-60 yrs old
• Signs:
Elevated Hb, WBC, Plt
MCV normal or microcytic
Splenomegaly (60%)

5. Presentation determined by:
Hyperviscosity and thrombosis
WBC turnover (gout)
Bruising, epistaxis, bleeding from GIT
Neurologic sx: vertigo, tinnitus, headache,
visual disturbances

6. PE
• Ruddy complexion
• Splenomegaly
• Cyanosis – R-L shunt  CHD
• Hypoxemia

7. Diagnosis
- detected incidentally after a routine FBC
RBC mass>125%
Leukocyte ALP high
EPO levels: normal or low
O2 saturation: normal

8. Treatment
Keep HCT <42% by phlebotomy
Hydoxyurea: (10-20mg/kg/d PO)
: used to control WBC/Plt

10. Prognosis
• Often remain well for decades

11. • Complications:
1. Extramedullary hematopoiesis
2. Myelofibrosis
3. Marrow failure/leukemia

12. http://crisbertcualteros.page.tl