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Polycythemia vera

Polycythemia vera. Morning Report 7/7/2010 Pahresah Roomiany. Differential Diagnoses. Factor V Leiden Prothrombin gene mutation Protein C/S deficiency Antithrombin deficiency Malignancy Antiphospholipid Ab syndrome Myeloproliferative disorders Paroxysmal nocturnal hemoglobinuria

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Polycythemia vera

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  1. Polycythemiavera Morning Report 7/7/2010 Pahresah Roomiany

  2. Differential Diagnoses • Factor V Leiden • Prothrombin gene mutation • Protein C/S deficiency • Antithrombin deficiency • Malignancy • Antiphospholipid Ab syndrome • Myeloproliferative disorders • Paroxysmal nocturnal hemoglobinuria • Nephrotic syndrome • Multiple myeloma • HIV/AIDS

  3. Types of polycythemia • Relative: due to decreased plasma volume • Secondary: # of erythrocytes increased. • Physiologically appropriate: • High altitude • COPD • R to L shunts • Carboxyhemoglobinemia • Cobalt ingestion • Physiologically inappropriate: • Renal vascular disease • Hepatic tumors • Renal cysts, transplant, RCC • Pheochromocytoma • Polycythemia vera: myeloproliferative disease

  4. Polycythemiavera • Increased production of RBCs and often wbc and platelets due to neoplastic disorder of hematopoetic stem cells. • Peak incidence in 6th/7th decades • Often found on routine blood counts • Slight male predominance

  5. Signs & Symptoms • “vasomotor” symptoms • Hyperviscosity: HA, dizziness, tinnitus, blurred vision • Thrombosis: transient visual disturbance (amaurosis, ocular migraine), Budd-chiari, erythromelalgia • Bleeding: easy bruising, epistaxis, GI bleed • Pruritus 2/2 to histamine release from basophils • PUD, gout (increased cell turnover)

  6. Diagnostic evaluation Suspect in patients with elevated RBC mass or elevated H/h with an arterial O2>92%, splenomegaly, thrombocytosis +/- leukocytosis, thrombotic complications, erythromelalgia or pruritus.

  7. Treatment • Phlebotomy: goal Hct <45% in men, <42% in women. • Microcytic anemia can cause increase in whole blood viscosity • Low dose ASA: decrease risk of cardiac event • Hydroxyurea: for high risk patients • Allopurinol for gout, H2 blocker/antihistamine for pruritus • JAK-2 inhibitors are showing positive treatment response in clinical trials5

  8. complications • Arterial, venous thrombosis • Evolution to AML due to cytotoxic agents (1-2%, a 100-fold increase in incidence in adult pop by age) • Remission with standard chemotherapy is low • 15-20% develop myeloid metaplasia • Replacement of marrow by fibrotic tissue • Movement of hematopoiesis into spleen and liver with worsening pancytopenia • Gout • Budd-chiari Syndrome

  9. PV and buddchiari • Hepatic venous outflow obstruction • Most cases of BCS in west are associated with MPD • Tx with TIPS, balloon angioplasty2 • High incidence of rethrombosis and graft failure in PV3 Fluoroscopic image of transjugular intrahepatic portosystemic shunt (TIPS) in progress. A catheter has been passed into the hepatic vein and a guidewire was passed into a portal vein branch. A stent has yet to be placed over the wire.

  10. Sources • Spivak, J. (2010). Narrative Review: thrombocytosis, polycythemia vera and JAK2 mutations: the phenotypic mimicry of chronic myeloproliferation. Ann Intern Med. 152:300-306. • Cruz, E. et.al. (2005).High incidence of recurrence and hematologic events following liver transplantation for budd-chiari syndrome. Clin transplant. 16: 501-506. • Buzas, C. (2009). Budd-chiari syndrome secondary to polycythemia vera. J Gastrointestin Liver Dis. 18: 363-366. • Rossi, D. (2007). Usefulness of JAK2V617F mutation in distinguishing idiopathic erythrocytosis from polycythemia vera. Leuk Res. 31: 97-101. • Finazzi, G. Barbui, T. (2008). Evidence and expertise in the management of polycythemia vera and essential thrombocythemia. Leukemia. 22: 1494-1502. • Tefferi, A and Vardiman JW. (2008) Classification and diagnosis of myeloproliferative neoplasms: The 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. • Kasper et.al. (16th Ed) Harrisons Principles of Internal Medicine.

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