POLYCYTHEMIA

POLYCYTHEMIA OR ERYTHROCYTOSIS
Is an excess of red blood cells characterized by a hematocrit higher than 55% Two major types:

Primary

secondary

PRIMARY POLYCYTHEMIA
Also called Polycythemia Vera Uncommon disorder of increased RBC production Commonly affects men of European Jewish ancestry between age 40 and 70

PATHOPHYSIOLOGY OF POLYCYTHEMIA VERA Neoplastic stem cell disorder: Overproduction of RBCs, WBCs and platelets
Colonies of endogenous erythroid stem cells develop Produce RBCs in the absence of erythropoietin Excess RBC production

SECONDARY POLYCYTHEMIA
Occurs in response to elevated erythropoietin levels Commonly is a compensatory response to hypoxia, often due to living at high altitude, smoking or chronic lung disease

RELATIVE POLYCYTHEMIA

Not due to an excess of RBCs but to fluid deficit. The total RBC count is normal, but fluid loss increases cell concentration, thus raising the hematocrit. Relative polycythemia is corrected by rehydration

RISK FACTORS
Hypoxia from long standing (chronic) lung disease and smoking Chronic carbon monoxide (CO) exposure People living at high altitudes People with genetic mutations and familial types of polycythemia and certain hemoglobin abnormalities

MANIFESTATIONS
Hypertension Headache, tinnitus, blurred vision Spleenomegaly ( polycythemia vera) Severe pruritus, extremity pain Plethora- ruddy, red color of the face, hands, feet and mucus membranes Weight loss, night sweats Gastrointestinal bleeding Intermittent claudication

TREATMENT

Phlebotomy (drawing blood or blood letting)

It

is the most essential part of the treatment. The recommended hematocrit of less than 45 in men and less than 42 in women is the goal of phlebotomy.
been recommended for some patients with primary polycythemia and higher risk of blood clot formation due to high blood viscosity.

Hydroxyurea (Hydrea)
has

TREATMENT

Aspirin and other anti-platelet agents (dipyridamole)

may

also be beneficial in patients with polycythemia by reducing clotting complications, unless the patient has a history of bleeding problems.

In patients with secondary polycythemia, the goal is to treat the underlying condition.

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