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Original article

Platelet-mediated erythromelalgic, cerebral, ocular and coronary microvascular ischemic and thrombotic manifestations in patients with essential thrombocythemia and polycythemia vera: A distinct aspirin-responsive and coumadin-resistant arterial thrombophilia

, MD, PhD, , , , , & show all
Pages 528-544 | Received 28 Nov 2005, Accepted 22 Mar 2006, Published online: 07 Jul 2009

References

  • Mitchell SW. On a rare vaso-motor neurosis of the extremities and on the maladies with which it may be confounded. Am J Med Sci 1878; 76: 2–36
  • Smith LA, Allen EV. Erythermalgia (erythromelalgia) of the extremities: A syndrome characterized by redness, heat and pain. Am Heart J 1938; 16: 175–188
  • Michiels JJ, Abels J, Steketee J, vanVliet HHDM, Vuzevski VD. Erythromelalgia caused by platelet-mediated arteriolar inflammation and thrombosis in thrombocythemia. Ann Intern Med 1985; 102: 466–471
  • Michiels JJ, Van Joost Th. Erythromelalgia and thrombocythemia. A causal relation. J Am Acad Dermatol 1990; 22: 107–111
  • Annetts DL, Tracy GD. Idiopathic thrombocythemia presenting with ischemia of the toes. Med J Austr 1966; 2: 180–182
  • Vreeken J, Van Aken WS. Spontaneous aggregation of blood platelets as a cause of idiopathic and recurrent painful toes and fingers. Lancet 1971; ii: 1394–1397
  • Preston FE, Emmanuel IG, Winfield DA, Malia RG. Essential thrombocythemia and peripheral gangrene. Br Med J 1974; 3: 548–552
  • Singh AK, Weitherley-Mein G. Microvascular occlusive lesions in primary thrombocythemia. Br J Haematol 1977; 36: 553–564
  • Redding KG. Thrombocythemia as a cause of erythromelalgia. Arch Dermatol 1977; 113: 468–471
  • Koudstaal P, Koudstaal A. Neurologic and visual symptoms in essential thrombocythemia: Efficacy of low-dose aspirin. Semin Thromb Hemost 1997; 23: 365–370
  • Jabaily J, Iland HJ, Laszlo J, Massey EW, Faguet GB, Brière J, Landaw A, Pisciotta AV. Neurologic manifestations of essential thrombocythemia. Ann Intern Med 1983; 99: 513–518
  • Michiels JJ, Ten Kate FWJ, Vuzevski VD, Abels J. Histopathology of erythromelalgia in thrombocythemia. Histopathology 1984; 8: 669–678
  • Van Genderen PJJ, Michiels JJ. Erythromelalgia: A pathognomonic microvascular thrombotic complication in essential thrombocythemia and polycythemia vera. Semin Thromb Hemostas 1997; 23: 357–363
  • Van Genderen PJJ, Michiels JJ, van Strik R, Lindemans J, van Vliet HHDM. Platelet consumption in thrombocythemia complicated by erythromelalgia: Reversal by aspirin. Thromb Haemost 1995; 73: 210–214
  • Van Genderen PJJ, Lucas IS, van Strik R, Vuzevski VD, Prins F, van Vliet HHDM, Michiels JJ. Erythromelalgia in essential thrombocythemia is characterized by platelet activation and endothelial cell damage but not by thrombin generation. Thromb Haemost 1996; 76: 333–338
  • Michiels JJ, Zijlstra FJ. Prostaglandin cyclooxygenase products but not thromboxane A2 is involved in the pathogenesis of erythromelalgia in thrombocythemia. Mediators Inflammation 1993; 2: 385–389
  • Van Genderen PJJ, Prins F, Michiels JJ, Schrör K. Thromboxane-dependent platelet activation in vivo precedes arterial thrombosis in thrombocythaemia: A rationale for the use of low-dose aspirin as an antithrombotic agent. Br J Haematol 1999; 104: 438–441
  • Michiels JJ. Normal life expectancy and thrombosis-free survival in aspirin treated essential thrombocythemia. Clin Appl Thromb Hemost 1999; 5: 30–36
  • Michiels JJ, Thiele J. Clinical and pathological criteria for the diagnosis of essential thrombocythemia, polycythemia vera and idiopathic myelofibrosis (agnogenic myeloid metaplasia). Int J Hematol 2002; 76: 133–145
  • Michiels JJ. Bone marrow histopathology and biological markers as specific clues to the differential diagnosis of essential thrombocythemia, polycythemia vera and prefibrotic or fibrotic myeloid metaplasia. Hematol J 2004; 5: 93–102
  • Lendrum AC, Fraser DS, Slidders W, Henderson R. Studies on the characteristics and staining of fibrin. J Clin Pathol 1962; 15: 401–413
  • Aster RH, Jandl JH. Platelet sequestration in man. J Clin Ivest 1964; 43: 843–849
  • Murphy EA, Francis ME. Estimation of blood platelet survival. The multiple hit model. Thromb Diathes Haemorrh 1971; 25: 53–80
  • Kutti J, Weinfeld A. Platelet survival in man. Scand J Haematol 1971; 8: 336–339
  • Kutti J, Weinfeld A. Platelet production and survival in polycythaemia vera with special reference to spleen size. Scand J Haematol 1972; 9: 97–112
  • Smith JB, Ingerman CM, Silver MJ. Malodialdehyde formation as an indicator of prostaglandin production by human platelets. J Lab Clin Med 1976; 88: 167–173
  • Crook D, Collins AJ. Comparison of effects of aspirin and indomethacin on human platelet prostaglandin synthethase. Ann Rheum Dis 1977; 36: 459–463
  • Huijgens PC, Van Den Berg CAM, Van Der Meer C, Imandt LMFM, Langenhuizen MMAC. Dosage of acetylsalicylic acid for inhibition of platelet function. Scand J Haematol 1980; 25: 76–80
  • Patrono C. Aspirin as an antiplatelet drug. New Engl J Med 1994; 330: 1287–1294
  • Schör K. Aspirin and platelets: The antiplatelet action of aspirin and its role in thrombosis treatment and prophylaxis. Semin thromb Hemost 1997; 23: 349–356
  • Landolfi R, Ciabattoni G, Patrihnani P, Castellana MAL, Pogliani E, Bizzi B, Patrono C. Increased thromboxane biosynthesis in patients with polycythemia vera: Evidence for aspirin-suppressible platelet activation in vivo. Blood 1992; 80: 1965–1971
  • Rocca B, Ciabattoni G, Tartaglione R, Cortelazzo S, Barbui T, Patrono C, Landolfi R. Increased thromboxane biosynthesis in essential thrombocythemia. Thromb Haemost 1995; 74: 1225–12230
  • Koudstaal PJ, Koudstaal A. Neurologic and visual symptoms in essential thrombocythemia: Efficacy of low-dose aspirin. Semn Thromb Hemost 1997; 23: 365–370
  • Michiels JJ, Koudstaal P, Mulder AH, van Vliet HHDM. Transient neurologic and ocular manifestations in primary thombocythemia. Neurology 1993; 43: 1107–1110
  • Michiels JJ, Van Genderen PJJ, Janssen PHP, Koudstaal P. Atypical transient ischemic attacks in thrombocythemia of various myeloproliferative disorders. Leukemia Lymphoma 1996; 22(Suppl 1)65–70
  • Scheffer MG, Michiels JJ, Simoons ML, Roelandt JRTC. Thrombocythemia and coronary artery disease. Am Heat J 1991; 22: 573–576
  • Fröhli P, Graf C, Rhyner K. Die Prophylaxe vasculärer Komplikationen bei Polycythaemia Vera und primärer Thrombozythämie mit niedrigdosierter Acetylsalicylsäure. Schweiz Med Wochenschr 1983; 113: 1622–1627
  • Van Genderen PJJ, Mulder PGH, Waleboer M, van de Moesdijk D, Michiels JJ. Prevention and treatment of thrombotic complications in essential thrombocythemia: Efficacy and safety of aspirin. Br J Haematol 1997; 97: 179–184
  • Michiels JJ. Aspirin and platelet-lowering agents for the prevention of vascular complications in essential thrombocythemia. Clin Appl Thromb Hemost 1999; 5: 247–251
  • Griesshammer M, Bangerter M, van Vliet HHDM, Michiels JJ. Aspirin in essential thrombocythemia: Status quo and quo vadis. Semin Thromb Hemost 1997; 23: 371–377
  • Berlin MI. Diagnosis and classification of the polycythemias. Semin Hematol 1975; 12: 339–351
  • Berk PD, Goldberg JD, Silverstein MN, Weinfeld A, Donovan PB, Ellis JT, Landauw SA, Laszlo J, Najean Y, Psciotta AV, Wasserman LR. Increased incidence of acute leukaemia in polycythemia vera associated with chlorambucil therapy. New Eng J Med 1981; 304: 441–447
  • Berk PD, Goldberg JD, Donovan PB, Fruchtman SM, Berlin NI, Wasserman LR. Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol 1986; 23: 132–143
  • Berk P, Wasserman LR, Fruchtman SM, Goldberg JD. Treatment of polycythemia vera: A summary of clinical trials conducted by the Polycythemia Vera study Group. Polycythemia vera and the myeloproliferative disorders, LR Wasserman, PD Berk, NI Berlin. WB Saunders, Philadelphia, PA 0000
  • Fruchtman SM, Mack K, Kaplan ME, Peterson P, Berk PD, Wasserman LR. From efficacy to safety: A Polycythemia Vera Study Group report on hydroxyurea in patients with polycythemia vera. Semin Hematol 1997; 34: 17–23
  • Messinezy M, Pearson TC, Pochazka A, Wetherley–Mein G. Treatment of primary proliferative polycythemia by venasection and low dose busulphan. Retrospective study from one centre. Br J Haematol 1985; 61: 657–666
  • Pearson TC, Wetherley-Mein G. Vascular occlusive episodes and venous hematocrit in primary proliferative polycythaemia. Lancet 1978; ii: 1219–1222
  • Thomas DJ, Marshall J, Russell RWR, Wetherley-Mein G, et al. Cerebral blood-flow in polycythemia. Lancet 1977; ii: 161–163
  • Thomas DJ, du Boullat GH, Marshall J, et al. Effect of haematocrit on cerebral blood flow in man. Lancet 1977; ii: 941–943
  • Michiels JJ, Berneman Z, Schroyens W, Urk H. Aspirin-responsive painful red blue, black toe or finger syndrome in plocythemia vera associated with thrombocythemia. Ann Hematol 2003; 82: 153–159
  • Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, Barbui T. Efficacy and safety of low-dose aspirin in polycythemia vera: Results of the ECLAP trial. New Engl J Med 2004; 350: 114–124
  • Budde U, Schaefer G, Mueller N, Egli H, Ruggeri ZM, Zimmerman TS. Acquired von Willebrand disease in the myeloproliferative syndrome. Blood 1984; 64: 981–985
  • Budde U, Dent JA, Berkovitz SD, Ruggeri ZM, Zimmerman TS. Subunit composition of plasma von Willebrand factor in patients with the myeloproliferative syndrome. Blood 1986; 68: 1213–1217
  • Fabris F, Casonato A, Del Ben MG, De Marco L, Girolami A. Abnormalities of von Willebrand factor in myeloproliferative disease: A relationship with bleeding diathesis. Br J Haematol 1986; 63: 75–83
  • Van Genderen PJJ, Michiels JJ, van der Poel-Luitgaarden SCAPM, van Vliet HHDM. Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: Relationship with platelet count. Ann Hematol 1994; 69: 81–84
  • Van Genderen PJJ, Budde U, Michiels JJ, van Strik R, van Vliet HHDM. The reduction of large von Willebrand factor multimers in plasma in essential thrombocythemia is related to the platelet count. Br J Hematol 1996; 93: 962–965
  • Michiels JJ. Acquired von Willebrand Disease due to increasing platelet count can readily explain the paradox of thrombosis and bleeding in thrombocythemia. Clin Appl Thromb Hemost 1999; 5: 147–151
  • Michiels JJ, Berneman Z, Schroyens W, Van Vliet HHDM. Pathophysiology and treatment of platelet-mediated microvascular disturbances, major thrombosis and bleeding complications in essential thrombocythaemia and polycythaemia vera. Platelets 2004; 15: 67–84
  • Michiels JJ. Erythromemalgia and thrombocythemia. Erasmus University, Rotterdam 1981, Thesis
  • Van Genderen PJJ, Michiels JJ. Erythromelalgic thrombotic and hemorrhagic thrombocythemia. Presse Med 1994; 23: 73–77

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