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Hamostaseologie 2003; 23(03): 138-143
DOI: 10.1055/s-0037-1619584
Original Article
Schattauer GmbH

Qualitative platelet defect and thrombohaemorrhagic complications in a patient with polycythaemia vera

Case 10Thrombose und Blutung als Komplikationen einer qualitativen Plättchenfunktionsstörung bei Polycythaemia vera
D. Friess
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
,
B. Lämmle
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
,
L. Alberio
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
› Author Affiliations
Further Information

Publication History

Publication Date:
23 December 2017 (online)

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Summary

We describe a 67-year-old patient with polycythaemia vera and pathological functional platelet studies. He not only suffered a transient ischaemic attack despite taking of antiplatelet agents, but also showed bleeding diathesis with cerebral bleeding and spontaneous suffusions. Platelet function studies and clinical findings improved after phlebotomy and cytoreductive treatment with hydroxyurea.

Thrombosis and haemorrhage account predominantly for morbidity and mortality in patients with polycythaemia vera. The pathophysiological mechanisms to explain thrombosis and bleeding in patients with myeloproliferative disorders including polycythaemia vera were intensively studied. However, up to now no clear correlation of laboratory findings in relation to clinical history of thrombosis and bleeding was demonstrated. In this report the most important pathophysiological mechanisms and therapy with antiplatelet agents are discussed.

Zusammenfassung

Ein 67-jähriger Patient mit Polycythaemia rubra vera erlitt trotz Einnahme eines Thrombozytenaggregationshemmers rezidivierende zerebrale Durchblutungsstörungen. Zusätzlich erlitt er eine zerebrale Blutung und wiederholte, spontane Suffusionen als Zeichen einer hämorrhagischen Diathese. Die Thrombozytenaggregationstests zeigten eindeutig eine Plättchenfunktionsstörung. Sowohl die Labor- wie auch die klinischen Befunde verbesserten sich nach Einleitung der Behandlung mit Aderlässen und Hydroxyurea. Thromboembolien und Blutungen sind hauptsächlich verantwortlich für Morbidität und Mortalität von Patienten mit Polycythaemia rubra vera.

Die pathophysiologischen Mechanismen, die Thrombose- und Blutungsneigung verursachen sollen, wurden in zahlreichen Studien untersucht. Eine eindeutige Korrelation zwischen den beschriebenen Laborbefunden und der klinischen Symptomatik konnte noch nicht gezeigt werden. In diesem Artikel werden die wichtigsten pathophysiologischen Mechanismen, u. a. im Hinblick auf die Behandlung mit Thrombozytenaggregationshemmern, diskutiert.

Keywords

Polycythaemia vera - platelet function - thrombosis - antiplatelet agents

Schlüsselwörter

Polyzythaemia rubra vera - Plättchenfunktionsstörung - Thrombose - Thrombozytenaggregationshemmer
 

  • References

  • 1 Ambrus JL, Ambrus CM, Dembinsky W. et al. Thromboembolic disease susceptibility related to red cell membrane fluidity in patients with polycythemia vera and effect of phlebotomies. J Med 1999; 30: 299-304.
    PubMedGoogle Scholar
  • 2 Ania BJ, Suman VJ, Sobell JL. et al. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989. Am J Hematol 1994; 47: 89-93.
    CrossrefPubMedGoogle Scholar
  • 3 Barbui T, Finazzi G. Risk factors and prevention of vascular complications in polycythemia vera. Semin Thromb Hemost 1997; 23: 455-61.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 4 Calzada C, Vericel E, Lagarde M. Low concentrations of lipid hydroperoxides prime human platelet aggregation specifically via cyclooxygenase activation. Biochem J 1997; 325: 495-500.
    CrossrefPubMedGoogle Scholar
  • 5 Cortelazzo S, Finazzi G, Ruggeri M. et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med 1995; 332: 1132-6.
    CrossrefPubMedGoogle Scholar
  • 6 De Stefano V, Teofili L, Leone G. et al. Spontaneous erythroid colony formation as the clue to an underlying myeloproliferative disorder in patients with Budd-Chiari syndrome or portal vein thrombosis. Semin Thromb Hemost 1997; 23: 411-8.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 7 Gruppo Italiano Studio Policitemia (GISP). Low-dose aspirin in polycythaemia vera: a pilot study. Br J Haematol 1997; 97: 453-6.
    CrossrefPubMedGoogle Scholar
  • 8 Gruppo Italiano Studio Policitemia. Polycythemia vera: the natural history of 1213 patients followed for 20 years. Ann Intern Med 1995; 123: 656-64.
    CrossrefPubMedGoogle Scholar
  • 9 Handa M, Watanabe K, Kawai Y. et al. Platelet unresponsiveness to collagen: involvement of glycoprotein Ia-IIa (alpha 2 beta 1 integrin) deficiency associated with a myeloproliferative disorder. Thromb Haemost 1995; 73: 521-8.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 10 Hankey GJ, Sudlow CL, Dunbabin DW. Thienopyridines or aspirin to prevent stroke and other serious vascular events in patients at high risk of vascular disease? A systematic review of the evidence from randomized trials. Stroke 2000; 31: 1779-84.
    CrossrefPubMedGoogle Scholar
  • 11 Hoffbrand AV, Lewis SM, Tuddenham EGD. Postgraduate Haematology. Fourth edition. New York: Arnold; 2001
    Google Scholar
  • 12 Holme S, Murphy S. Platelet abnormalities in myeloproliferative disorders. Clin Lab Med 1990; 10: 873-88.
    PubMedGoogle Scholar
  • 13 Landolfi R, Ciabattoni G, Patrignani P. et al. Increased thromboxane biosynthesis in patients with polycythemia vera: evidence for aspirin-suppressible platelet activation in vivo. Blood 1992; 80: 1965-71.
    PubMedGoogle Scholar
  • 14 Landolfi R, Marchioli R, Patrono C. Mechanisms of bleeding and thrombosis in myeloproliferative disorders. Thromb Haemost 1997; 78: 617-21.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 15 Landolfi R, Marchioli R. European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP): a randomized trial. Semin Thromb Hemost 1997; 23: 473-8.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 16 Landolfi R, Rocca B, Patrono C. Bleeding and thrombosis in myeloproliferative disorders: mechanisms and treatment. Crit Rev Oncol Hematol 1995; 20: 203-22.
    CrossrefPubMedGoogle Scholar
  • 17 Landolfi R. Bleeding and thrombosis in myeloproliferative disorders. Curr Opin Hematol 1998; 5: 327-31.
    CrossrefPubMedGoogle Scholar
  • 18 Le Blanc K, Lindahl T, Rosendahl K. et al. Impaired platelet binding of fibrinogen due to a lower number of GPIIB/IIIA receptors in polycythemia vera. Thromb Res 1998; 91: 287-95.
    CrossrefPubMedGoogle Scholar
  • 19 Malpass TW, Savage B, Hanson SR. et al. Correlation between prolonged bleeding time and depletion of platelet dense granule ADP in patients with myelodysplastic and myeloproliferative disorders. J Lab Clin Med 1984; 103: 894-904.
    PubMedGoogle Scholar
  • 20 Messinezy M, Pearson TC. The classification and diagnostic criteria of the erythrocytoses (polycythaemias). Clin Lab Haematol 1999; 21: 309-16.
    CrossrefPubMedGoogle Scholar
  • 21 Murphy S. Therapeutic dilemmas: balancing the risks of bleeding, thrombosis, and leukemic transformation in myeloproliferative disorders (MPD). Thromb Haemost 1997; 78: 622-6.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 22 Pearson TC. Hemorheologic considerations in the pathogenesis of vascular occlusive events in polycythemia vera. Semin Thromb Hemost 1997; 23: 433-9.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 23 Ravandi-Kashani F, Schafer AI. Microvascular disturbances, thrombosis, and bleeding in thrombocythemia: current concepts and perspectives. Semin Thromb Hemost 1997; 23: 479-88.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 24 Schafer AI. Deficiency of platelet lipoxygenase activity in myeloproliferative disorders. N Engl J Med 1982; 306: 381-6.
    CrossrefPubMedGoogle Scholar
  • 25 Sharis PJ, Cannon CP, Loscalzo J. The antiplatelet effects of ticlopidine and clopidogrel. Ann Intern Med 1998; 129: 394-405.
    CrossrefPubMedGoogle Scholar
  • 26 Tartaglia AP, Goldberg JD, Berk PD. et al. Adverse effects of antiaggregating platelet therapy in the treatment of polycythemia vera. Semin Hematol 1986; 23: 172-6.
    PubMedGoogle Scholar
  • 27 Tefferi A, Solberg LA, Silverstein MN. A clinical update in polycythemia vera and essential thrombocythemia. Am J Med 2000; 109: 141-9.
    CrossrefPubMedGoogle Scholar
  • 28 Tefferi A. Chronic myeloid disorders: Classification and treatment overview. Semin Hematol 2001; 38: 1-4.
    PubMedGoogle Scholar
  • 29 van Genderen PJ, Michiels JJ, van Strik R. et al. Platelet consumption in thrombocythemia complicated by erythromelalgia: reversal by aspirin. Thromb Haemost 1995; 73: 210-4.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 30 van Genderen PJ, Troost MM. Polycythaemia vera and essential thrombocythaemia in the elderly. Drugs Aging 2000; 17: 107-19.
    CrossrefPubMedGoogle Scholar
  • 31 Weber AA, Braun M, Hohlfeld T. et al. Recovery of platelet function after discontinuation of clopidogrel treatment in healthy volunteers. Br J Clin Pharmacol 2001; 52: 333-6.
    CrossrefPubMedGoogle Scholar
  • 32 Wehmeier A, Sudhoff T, Meierkord F. Relation of platelet abnormalities to thrombosis and hemorrhage in chronic myeloproliferative disorders. Semin Thromb Hemost 1997; 23: 391-402.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 33 Willoughby S, Pearson TC. The use of aspirin in polycythaemia vera and primary thrombocythaemia. Blood Rev 1998; 12: 12-22.
    CrossrefPubMedGoogle Scholar