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Polycythemia Vera (lots of red cells - for real)

Polycythemia Vera (lots of red cells - for real). An 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.

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Polycythemia Vera (lots of red cells - for real)

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  1. Polycythemia Vera(lots of red cells - for real) • An 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

  2. Definition of Erythrocytosis • Normal hematocrit at FMLH: • Male 47  5 percent • Female 42  5 percent • Normal hemoglobin at FMLH: • Male 15  2 gm/dl • Female 13.5  1.5 gm/dl

  3. Absolute vs. Relative Erythrocytosis

  4. RBC Mass - 51Chromium Assay

  5. Pathophysiology of Polycythemia

  6. Secondary Polycythemia • Appropriate EPO (tissue/kidney hypoxia) • pulmonary disease • high altitude • congenital heart disease • abnormal hemoglobin • high affinity • carboxyhemoglobin

  7. Secondary Polycythemia • Inappropriate EPO (ectopic production) • Tumors (hepatoma, renal carcinoma, leiomyoma, hamartoma) • Renal disorders (transplantation, cysts) • hemangiomas • Androgen abuse • EPO abuse • Familial polycythemia

  8. Polycythemia Vera • P. vera is a rare disease • Median age 60 - 65 years • Clinical features • Attributed to increased blood viscosity and poor oxygen delivery to organs (brain) • Poor O2 delivery leads to ischemia and thrombosis • Expanded blood volume and viscosity leads to increased cardiac work load

  9. Oxygen delivery vs. Hematocrit J Clin Invest 1963;42:1150

  10. Symptoms Headache Weakness Pruritis (aquagenic) Dizziness Diaphoresis Visual disturbance Weight loss Signs Splenomegaly 70% Skin plethora 67% Hepatomegaly 40% Conjunctival plethora 59% Systolic Hypertension 72% P. Vera - Symptoms & Signs

  11. Criteria RBC mass elevated SaO2 > 92% Splenomegaly (or) thrombocytosis Leukocytosis high LAP high B12 Significance True vs. spurious R/O most 2 causes Evidence for MPD False Positive 0.5% smokers, drinkers P. Vera - Diagnosis (PVSG criteria)

  12. P. vera - Bone Marrow Biopsy

  13. P. Vera - Natural History

  14. Treatment - PVSG • Founded 1967 • Protocol 01 • Phlebotomy vs. Chlorambucil vs. 32P • Protocol 05 • Phlebotomy with ASA, dipyridamole vs. 32P • Protocol 08 • Phlebotomy vs. Hydroxyurea

  15. Risk of Thrombosis from Treatment(PVSG 01) * p = 0.015

  16. Types of Thrombosis(PVSG 01)

  17. Risk of Cancer from Treatment(PVSG 01) * p < 0.01

  18. PVSG 08 - Hydroxyurea

  19. Advantages quick, easy less trips to clinic low risk of cancer no medication need compliance Disadvantages thrombosis risk symptoms of iron deficiency perhaps faster to “spent phase” vascular access cardiovascular effects no effect on spleen no effect on platelets Treatment Options - Phlebotomy

  20. Advantages quick and effective thrombosis risk low no medication follow-up need minimal compliance easier reduces spleen size lowers all counts few side-effects Disadvantages risk of leukemia uncontrolled effects childbearing risk radiation issues Treatment Options - 32P

  21. Advantages quick and effective thrombosis risk low reduces spleen size lowers all counts leukemia risk low few side-effects Disadvantages close monitoring childbearing risk compliance (daily medication) GI toxicity (rare) leukemia risk (?) Treatment Options - Hydroxyurea

  22. Treatment Options - Summary

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