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uncommon disorder - distinguish from other causes of erythrocytosis Diagnosis depends on knowledge of erythropoeisis Complications most commonly from thrombosis and vascular incidents Long natural history with treatment
Normal
hematocrit at FMLH:
hemoglobin at FMLH:
Normal
Spurious
Polycythemia
28 ml/kg
> 36 ml/kg
Appropriate
Inappropriate
hamartoma) Renal disorders (transplantation, cysts) hemangiomas Androgen abuse EPO abuse Familial polycythemia
P.
oxygen delivery to organs (brain) Poor O2 delivery leads to ischemia and thrombosis Expanded blood volume and viscosity leads to increased cardiac work load
180 160
Oxygen Transport
60
80
Symptoms
Signs
Weight loss
Criteria
Significance
False
Positive 0.5%
smokers, drinkers
Founded
1967 Protocol 01
Phlebotomy vs. Chlorambucil vs.
Protocol Protocol
32P
05
32P
08
Event
CVA Venous MI P. arterial Pulm. Infarct
Percent
35% 26% 12% 9% 6%
Treatment Phlebotomy
7 years 1.29
Chlorambucil 2.00*
32
1.88*
Treatment
Hydroxyurea
(n = 51)
Thrombosis
22% 37%
Leukemia
6% 2%
Phlebotomy
(n = 134)
Advantages
Disadvantages
quick, easy less trips to clinic low risk of cancer no medication need compliance
deficiency perhaps faster to spent phase vascular access cardiovascular effects no effect on spleen no effect on platelets
Advantages
Disadvantages
quick and effective thrombosis risk low no medication follow-up need minimal compliance easier reduces spleen size
Advantages
Disadvantages
quick and effective thrombosis risk low reduces spleen size lowers all counts leukemia risk low few side-effects