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POLYCYTHEMIA

Polycythemia
Spurious polycythemia (elevated red cell mass
due to contracted blood volume)

Secondary polycythemia
Hypoxia (high altitude, cardiac or pulmonary disease) Carboxyhemoglobin (smoking) Kidney lesions (polycystic kidney disease) Erythropoeitin secreting tumours (hepatoma)

Polycythemia vera

Polythemia rubra vera (PRV)


A myeloproliferative disorder characterised by autonomous overproduction of erythroid cells Erythroid production is independent of erythropoeitin A mutation in JAK2 seen in 95% cases Rare before the age of 40 years Men > Women

PRV (clinical features)


Incidental finding Symptoms of hyperviscosity & expanded blood volume
Lassitude Loss of concentration Headaches Dizziness Blackouts

PRV (clinical features)


Symptoms of hyperviscosity & expanded blood volume
Blurred vision Tinnitus Pruritis after a warm shower Epistaxis

PRV (clinical features)


Thrombotic manifestations of ;
Peripheral arterial disease Coronary artery disease Cerebrovascular accident

Peptic ulcer Gout SIGNS


Plethora Splenomegaly

PRV (diagnosis)
Complete blood count
Elevated counts of red cells, white cells & platelets High hemoglobin Elevated hematocrit Normal morphology of blood cells

Low serum erythropoeitin JAK2 mutation High uric acid

PRV (Management)
Venesection
Prompt relief of hyperviscosity symptoms Usual volume of blood removed is 400-500 mL Repeated every 5-7 days Target hematocrit is 45%

Aspirin to prevent thrombosis Drugs


Hydroxycarbamide Interferon

Radioactive phosphorus

PRV (Prognosis)
Median survival in treated patients > 10 years Major causes of morbidity & mortality are cerbrovascular or coronary diseases PRV may convert to another type of myeloproliferative disorder