Polycythemia vera is a myeloproliferative disorder characterized by increased red cell mass and frequently complicated by venous and arterial thrombosis. The mechanism underlying the increased incidence of thrombotic events remains illusive. Presented in this report are a case of a 77-year-old man diagnosed with polycythemia vera and a review of the current literature on the mechanisms underlying the increased incidence of thrombotic events in polycythemia vera.
Get full access to this article
View all access options for this article.
References
1.
Vaquez MH: Sur une forme speciale de cyanose s'accompagnant d'hyperglobulie excessive et persistanteCompt Rend Soc D Biol44:384, 1892.
2.
Osler W.: Chronic cyanosis with polycythemia and enlarged spleen: A new clinical entityAm J Med Sci126:187-201, 1903.
3.
Cievitz E., Thiede T.: Complications and causes of death in polycythemia veraActa Med Scand172:513-523, 1962.
4.
Rosenthal N. , Bassen FA: Course of polycythemiaArch Intern Med62:903-917, 1938.
5.
Berlin NI: Diagnosis and classification of the polycythemiasSemin Hematol12:339-351, 1975.
Gruppo Italiano Studio Policitemia (GISP) : Polycythemia vera: The natural history of 1213 patients followed over 20 yearsAnn Intern Med123:656-664, 1995.
8.
Pearson TC: Hemorheological considerations in the pathogenesis of vascular occlusive events in polycythemia veraSemin Thromb Hemost23:433-439, 1997.
9.
Berger S., Aledort LM, Gilgert HS, et al: Abnormalities of platelet function in patients with polycythemia veraCancer Res33:2683-2687, 1973.
10.
Wehmeier A. , Fricke S., Scharf RE, et al: A prospective study of haemostatic parameters in relation to the clinical course of myeloproliferative disordersEur J Haematol45:191-197, 1990.
11.
Mehta P., Mehta J., Ross M., et al: Decreased platelet aggregation but increased thromboxane A2 generation in polycythemia veraArch Intern Med145:1225-1227, 1985.
12.
Landolfi R. , Ciabattoni G., Patrignani P., et al: Increased thromboxane biosynthesis in patients with polycythemia vera: Evidence for aspirin-suppressible platelet activation in vivoBlood80:1965-1971, 1992 .
13.
Van Genderen PJJ, Prins FJ, Michiels JJ, et al: Thromboxane-dependent platelet activation in vivo precedes arterial thrombosis in thrombocythaemia: A rationale for the use of low-dose aspirin as an antithrombotic agentBr J Haematol104:438-441, 1999.
14.
Gruppo Italiano Studio Policitemia (GISP) : Low-dose aspirin in polycythaemia vera: A pilot studyBr J Haematol97:453-456, 1997.
15.
Landolfi R. , Marchioli R.: European collaboration on low-dose aspirin in polycythemia vera (ECLAP): A randomized trialSemin Thromb Hemost23:473-478, 1997.
16.
Carvalho A. , Ellman L.: Activation of the coagulation system in polycythemia veraBlood47:669-678, 1976.
17.
Kornberg A. , Rahimi-Levene N., Yona R., et al: Enhanced generation of monocyte tissue factor and increased plasma prothrombin fragment 1+2 levels in patients with polycythemia vera: Mechanism of activation of blood coagulationAm J Hematol56:5-11,1997.
18.
Lamparter S. , Schuermann M., Heidtmann HH: APC resistance as an additional thrombotic risk factor in a patient suffering from polycythemia vera and recurrent thrombosisAnn Hematol74:49-50, 1997.
19.
Rintelen C. , Mannhalter C., Pabinger I., et al: Factor V Leiden mutation is not a major cause of thrombotic tendency in essential thrombocythaemia (ET) and polycythemia vera (PV) [abstract]Onkologie18(S2):153, 1995.
20.
Zoppi M., Furlan M., Brun del Re G., et al : Decreased free protein S levels in polycythemia veraThromb Haemost64:177-178, 1990.
21.
Bucalossi A. , Marotta G., Bigazzi C., et al: Reduction of antithrombin III, protein C and protein S levels and activated protein C resistance in polycythemia vera and essential thrombocythemia patients with thrombosisAm J Hematol52:14-20, 1996.
22.
Cancelas JA , Garcia-Avello A., Garcia-Frade LJ: High plasma levels of plasminogen activator inhibitor (PAI-1) in polycythemia vera and essential thrombocythemia are associated with thrombosisThromb Res75:513-520, 1994.
23.
Posan E., Ujj G., Kiss A., et al: Reduced in vitro clot lysis and release of more active platelet PAI-1 in polycythemia vera and essential thrombocythemiaThromb Res90:51-56, 1998.
24.
Tonon G., Boyd K., Lecchi A., et al: Plasma homocysteine levels in 10 patients with polycythemiaHaematologica82:343-344, 1997.
25.
Gisolanger H., Rodeghier F., Ruggeri M., et al: Homocysteine levels in polycythemia vera and essential thrombocythemiaBr J Haematol105:551-555, 1999.
26.
LeBlanc K., Samuelsson J.: Elevated plasma lipoprotein (a)—a potential risk factor for thrombotic complications in polycythemia veraEuro J Haematol571:257-258, 1996.
