Management

1. Management

2. Phlebotomy • Phlebotomy or bloodletting has been the mainstay of therapy • Remove excess cellular elements to improve the circulation of blood by lowering the blood viscosity • mainly red blood cells Harrison’s Principle of Internal Medicine 17thed http://emedicine.medscape.com/article/205114-treatment Jan 23, 2009

3. Phlebotomy • Patients with hematocrit values of less than 70% may be bled twice a week to reduce the hematocrit to the range of less than 45% • Patients with severe plethora who have altered mentation or associated vascular compromise can be bled more vigorously, with daily removal of 500 mL of whole blood Harrison’s Principle of Internal Medicine 17thed http://emedicine.medscape.com/article/205114-treatment Jan 23, 2009

4. Post-Phlebotomy • volume replacement with saline solution after each procedure to avoid postural hypotension • use myelosuppressive agents (Hydroxyurea) to avoid thrombotic or hemorrhagiccomplications http://emedicine.medscape.com/article/205114-treatment Jan 23, 2009

5. Hydroxyurea • effective agent for myelosuppression • Reduced the risk of thrombosis compared with phlebotomy alone and should be the drug of choice for patients older than 40 years • however, concerns have been raised regarding long-term risks for leukemic transformation Harrison’s Principle of Internal Medicine 17thed http://emedicine.medscape.com/article/205114-treatment Jan 23, 2009

6. Anagrelide (Agrylin) • A cyclic adenosine monophosphate phosphodiesterase inhibitor that prevents platelet aggregation and inhibits megakaryocyte maturation, thereby decreasing platelet counts • To date, this agent does not appear to increase the risk of acute leukemia in patients with PV and ET over time http://emedicine.medscape.com/article/205114-treatment Jan 23, 2009