Polycythemia Vera (lots of red cells - for

Polycythemia Vera (lots of red cells - for

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 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

Absolute vs. Relative Erythrocytosis Plasma Vol RBC Normal Spurious Polycythemia RBC Mass - 51Chromium Assay Female RBC Plasma

Total Blood Vol 25 ml/kg 35 ml/kg 60 ml/kg 33 ml/kg 61 ml/kg > 32 ml/kg Male 28 ml/kg

> 36 ml/kg Pathophysiology of Polycythemia Secondary Polycythemia Appropriate EPO (tissue/kidney hypoxia) pulmonary disease high altitude congenital heart disease abnormal hemoglobin high affinity carboxyhemoglobin Secondary Polycythemia

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

Oxygen delivery vs. Hematocrit Oxygen Transport 180 160 140 120 100 80 60 40 20 0 0 20 40

60 80 Hct J Clin Invest 1963;42:1150 P. Vera - Symptoms & Signs 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 - Diagnosis (PVSG criteria) 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 - Bone Marrow Biopsy P. Vera - Natural History Thrombosis/embolism AML Other cancer Hemorrhage Myelofibrosis Other PVSG 31% 19% 15%

6% 4% 25% GISP 30% 15% 16% 3% 3% 35% Treatment - PVSG Founded 1967 Protocol 01 Phlebotomy vs. Chlorambucil vs. 32P Protocol 05 Phlebotomy with ASA, dipyridamole vs. 32P

Protocol 08 Phlebotomy vs. Hydroxyurea Risk of Thrombosis from Treatment (PVSG 01) Treatment 3 years Overall Phlebotomy 23% 38%* Chlorambucil

10% 30% 32 13% 34% P * p = 0.015 Types of Thrombosis (PVSG 01) Event

Percent CVA 35% Venous 26% MI 12% P. arterial 9% Pulm. Infarct

6% Risk of Cancer from Treatment (PVSG 01) Treatment 7 years 14 years Phlebotomy 1.29 1.49 Chlorambucil 2.00*

2.38* 32 1.86* P 1.88* * p < 0.01 PVSG 08 - Hydroxyurea Treatment Hydroxyurea Thrombosis Leukemia

22% 6% 37% 2% (n = 51) Phlebotomy (n = 134) Treatment Options - Phlebotomy 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 - 32P 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 - Hydroxyurea 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 - Summary

P . V e ra P h le b o t o m iz e t o H C T < 4 5 Age > 70 H y d ro x y u re a 32P? Age 50 - 70 H y d ro x y u re a P h le b o t o m y Age < 50 P h le b o t o m y H y d ro x y u re a

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