Semin Thromb Hemost 2006; 32(4): 430-436
DOI: 10.1055/s-2006-942764
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Update on Diagnosis and Management of Essential Thrombocythemia

Heinz Gisslinger1
  • 1Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria
Further Information

Publication History

Publication Date:
29 June 2006 (online)

ABSTRACT

Patients with essential thrombocythemia carry a high risk for thromboembolic and bleeding events but they have an almost normal life expectancy. A careful evaluation of the medical history and an exact diagnosis is mandatory to estimate each patient's risk for morbidity and to choose the most appropriate treatment measure. In patients with the need of cytoreductive therapy, the benefits of therapy have to outweigh the potential risks of drug toxicities. Hydroxyurea is the most useful cytoreductive drug for elderly patients; in younger persons, interferon α or anagrelide may be the drugs of choice. The combination of anagrelide with acetylsalicylic acid may be contraindicated in patients with a history of bleeding.

REFERENCES

  • 1 Thiele J, Kvasnicka H M. A critical reappraisal of the WHO classification of the chronic myeloproliferative disorders.  Leuk Lymphoma. 2006;  47 381-396
  • 2 Ruggeri M, Tosetto A, Frezzato M, Rodeghiero F. The rate of progression to polycythemia very or essential thrombocythemia in patients with erythrocytosis or thrombocytosis.  Ann Intern Med. 2003;  139 470-475
  • 3 Passamonti F, Rumi E, Pungolino E et al.. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia.  Am J Med. 2004;  117 755-761
  • 4 Ania B J, Suman V J, Sobell J L, Codd M B, Silverstein M N, Melton III L J. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989.  Am J Hematol. 1994;  47 89-93
  • 5 Mesa R A, Silverstein M N, Jacobsen S J, Wollan P C, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995.  Am J Hematol. 1999;  61 10-15
  • 6 Rozman C, Giralt M, Feliu E, Rubio D, Cortes M T. Life expectancy of patients with chronic nonleukemic myeloproliferative disorders.  Cancer. 1991;  67 2658-2663
  • 7 Jensen M K, de Nully Brown P, Nielsen O J, Hasselbalch H C. Incidence, clinical features and outcome of essential thrombocythaemia in well defined geographical area.  Eur J Haematol. 2000;  65 132-139
  • 8 Griesshammer M, Bangerter M, Sauer T, Wennauer R, Bergmann L, Heimpel H. Aetiology and clinical significance of thrombocytosis: analysis of 732 patients with an elevated platelet count.  J Intern Med. 1999;  245 295-300
  • 9 Tefferi A, Pardanani A. Mutation screening for JAK2V617F: When to order the test and how to interpret the results.  Leuk Res. 2006;  , In press
  • 10 Thiele J, Kvasnicka H M. Chronic myeloproliferative disorders with thrombocythemia: a comparative study of two classification systems (PVSG, WHO) on 839 patients.  Ann Hematol. 2003;  82 148-152
  • 11 Cortelazzo S, Viero P, Finazzi G, D'Emilio A, Rodeghiero F, Barbui T. Incidence and risk factors for thrombotic complications in a historical cohort of 100 patients with essential thrombocythemia.  J Clin Oncol. 1990;  8 556-562
  • 12 Besses C, Cervantes F, Pereira A et al.. Major vascular complications in essential thrombocythemia: a study of the predictive factors in a series of 148 patients.  Leukemia. 1999;  13 150-154
  • 13 Jensen M K, de Nully Brown P, Lund B V, Nielsoen O J, Hasselbalch H C. Increased platelet activation and abnormal membrane glycoprotein content and redistribution in myeloproliferative disorders.  Br J Haematol. 2000;  110 116-124
  • 14 Cardin F, Graffeo M, McCormick P A, McIntyre N, Burroughs A. Adult “idiopathic” extrahepatic venous thrombosis. Importance of putative “latent” myeloproliferative disorders and comparison with cases with known etiology.  Dig Dis Sci. 1992;  37 335-339
  • 15 Denninger M H, Chait Y, Casadevall N et al.. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors.  Hepatology. 2000;  31 587-591
  • 16 Gisslinger H, Muellner M, Pabinger I et al.. Mutation of the prothrombin gene and thrombotic events in patients with polycythemia vera or essential thrombocythemia: a cohort study.  Haematologica. 2005;  90 408-410
  • 17 Regev A, Stark P, Blickstein D, Lahav M. Thrombotic complications in essential thrombocythemia with relatively low platelet counts.  Am J Hematol. 1997;  56 168-172
  • 18 Michiels J J, Koudstaal P J, Mulder A H, van Vliet H H. Transient neurologic and ocular manifestations in primary thrombocythemia.  Neurology. 1993;  43 1107-1110
  • 19 Michiels J J. Platelet-mediated microvascular inflammation and thrombosis in thrombocythemia vera: a distinct aspirin-responsive arterial thrombophilia, which transforms into a bleeding diathesis at increasing platelet counts.  Pathol Biol (Paris). 2003;  51 167-175
  • 20 Kessler C M, Klein H G, Havlik R J. Uncontrolled thrombocytosis in chronic myeloproliferative disorders.  Br J Haematol. 1982;  50 157-167
  • 21 Fenaux P, Simon M, Caulier M T, Lai J L, Goudemand J, Bauters F. Clinical course of essential thrombocythemia in 147 cases.  Cancer. 1990;  66 549-556
  • 22 Jantunen R, Juvonen E, Ikkala E, Oksanen K, Anttila P, Ruutu T. The predictive value of vascular risk factors and gender for the development of thrombotic complications in essential thrombocythemia.  Ann Hematol. 2001;  80 74-78
  • 23 Cortelazzo S, Finazzi G, Ruggeri M et al.. Hydroxyurea for patients with essential thrombocythemia and a high risk thrombosis.  N Engl J Med. 1995;  332 1132-1136
  • 24 Gisslinger H, Chott A, Scheithauer W, Gilly B, Linkesch W, Ludwig H. Interferon in essential thrombocythaemia.  Br J Haematol. 1991;  79(suppl) 42-47
  • 25 Steurer M, Gastl G, Jedrzejczak W W et al.. Anagrelide for thrombocytosis in myeloproliferative disorders: a prospective study to assess efficacy and adverse event profile.  Cancer. 2004;  101 2239-2246
  • 26 van Genderen P J, Prins F J, Lucas I S et al.. Decreased half-life time of plasma von Willebrand factor collagen binding activity in essential thrombocythaemia: normalization after cytoreduction of the increased platelet count.  Br J Haematol. 1997;  99 832-836
  • 27 Elliott M A, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia.  Br J Haematol. 2004;  128 275-290
  • 28 Sabatine M S, Morrow D A, Cannon C P et al.. Relationship between baseline white blood cell count and degree of coronary artery disease and mortality in patients with acute coronary syndromes: a TACTICS-TIMI 18 (Treat Angina with Aggrastat and determine Cost of Therapy with an Invasive or Conservative Strategy-Thrombolysis in Myocardial Infarction 18 trial) substudy.  J Am Coll Cardiol. 2002;  40 1761-1768
  • 29 Falanga A, Marchetti M, Evangelista V et al.. Polymorphonuclear leukocyte activation and hemostasis in patients with essential thrombocythemia and polycythemia vera.  Blood. 2000;  96 4261-4266
  • 30 Neunteufl T, Heher S, Stefenelli T, Pabinger I, Gisslinger H. Endothelial dysfunction in patients with polycythaemia vera.  Br J Haematol. 2001;  115 354-359
  • 31 Jensen M K, de Nully Brown P, Lund B V, Nielsen O J, Hasselbalch H C. Increased circulating platelet-leukocyte aggregates in myeloproliferative disorders is correlated to previous thrombosis, platelet activation and platelet count.  Eur J Haematol. 2001;  66 143-151
  • 32 Ruggeri M, Gisslinger H, Tosetto A et al.. Factor V Leiden mutation carriership and venous thromboembolism in polycythemia vera and essential thrombocythemia.  Am J Hematol. 2002;  71 1-6
  • 33 Gisslinger H, Rodeghiereo F, Ruggeri M et al.. Homocysteine levels in polycythaemia vera and essential thrombocythaemia.  Br J Haematol. 1999;  105 551-555
  • 34 Harrison C N, Gale R E, Machin S J, Linch D C. A large proportion of patients with a diagnosis of essential thrombocythemia do not have a clonal disorder and may be at lower risk of thrombotic complications.  Blood. 1999;  93 417-424
  • 35 Griesshammer M, Klippel S, Strunck E et al.. PRV-1 mRNA expression discriminates two types of essential thrombocythemia.  Ann Hematol. 2004;  83 364-370
  • 36 Kralovics R, Passamonti F, Buser A S et al.. A gain-of-function mutation of JAK2 in myeloproliferative disorders.  N Engl J Med. 2005;  352 1779-1790
  • 37 Barbui T, Barosi G, Grossi A et al.. Practice guidelines for the therapy of essential thrombocythemia. A statement from the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation.  Haematologica. 2004;  89 215-232
  • 38 Harrison C N, Campbell P J, Buck G et al.. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia.  N Engl J Med. 2005;  353 33-45

 University Professor
 Dr.
Heinz Gisslinger

Medical University of Vienna, Department of Internal Medicine I, Division of Hematology and Blood Coagulation

Währinger-Gürtel 18-20, A-1090 Vienna, Austria

Email: heinz.gisslinger@meduniwien.ac.at

    >