Surgical interventions in patients with myeloproliferative disease – prophylaxis and treatment of haemostatic disorders
Authors:
P. Ďulíček; J. Voglová; J. Malý
Authors‘ workplace:
Oddělení klinické hematologie II. interní kliniky Lékařské fakulty UK a FN Hradec Králové, přednosta prof. MUDr. Jaroslav Malý, CSc.
Published in:
Vnitř Lék 2010; 56(1): 61-66
Category:
Reviews
Overview
Despite many decades of laboratory and clinical research of pathogenesis bleeding and thrombosis and the search for the identification of the risk factors, the accomplished results are unsatisfactory in clinical praxis. In our article, we discuss incidence, pathogenesis, clinical manifestation and the risk factors for coagulopathy in patients with myeloproliferative disease. The surgical procedures are associated with higher risk of morbidity and mortality. The aim of the article is the formulation of the recommendations concerning management of surgical interventions with the goal to reduce the risk of bleeding and thrombotic diathesis.
Key words:
myeloproliferative disorders – thrombosis – bleeding – surgical interventions
Sources
1. Schafer AI. Bleeding and thrombosis in the myeloproliferative disorders. Blood 1984; 64: 1–12.
2. Landolfi R, Rocca B, Patron C. Bleeding and thrombosis in myeloproliferative disorders: mechanism and treatment. Crit Rev Oncol Hematol 1995; 20: 203–222.
3. Elliot MA, Tefferi A. Thrombosis and haemorrhage in polycythemia vera and essential thrombocythaemia. Br J Haematol 2004; 128: 275–290.
4. Cortelazzo S, Viero P, Finazzi G et al. Incidence and risk factors for thrombotic complications in a historical cohort of 100 patients with essential thrombocythemia. J Clin Oncol 1990; 8: 556–562.
5. Jensen MK, de Nully Brown P, Nielsen OJ et al. Incidence, clinical features and outcome of essential thrombocythaemia in a well defined geographical area. Eur J Haematol 2000; 65: 132–139.
6. Landolfi R, Cipriani MC, Novarese L. Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: pathogenic mechanism and prevention. Best Pract Res Clin Haematol 2006; 19: 617–633.
7. Pearson TC. Hemorhelologic considerations in the pathogenesis of vascular occlusive events in polycythemia vera. Semin Thromb Hemost 1997; 23: 433–439.
8. Regev A, Stark P, Blickstein D et al. Thrombotic complications in essential thrombocythemia with relatively low platelet counts. Am J of Haematol 1997; 56: 168–172.
9. Storen EC, Tefferi A. Long‑term use of anagrelide in young patients with essential thrombocythemia. Blood 2001; 97: 863–866.
10. Cortelazzo S, Finazzi G, Ruggeri M et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J of Med 1995; 332: 1132–1136.
11. Michiels JJ, Budde U, van der Planken M et al. Acquired von Willebrand syndromes: clinical features, etiology, pathophysiology, classification and management. Bailliereś Best Pract Clin Heamatol 2001; 14: 401–436.
12. Michiels JJ, Berneman Z, Schroyens W et al. 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 coumadine‑resistant arterial thrombophilia. Platelets 2006; 17: 528–544.
13. Wehmeier A, Fricke S, Scharf RE et al. A prospective study of haemostatic parameters in relation to the clinical course of myeloproliferative disorders. Eur J Haematol 1990; 45: 191–197.
14. Schafer AI. Deficiency of platelet lipoxygenase aktivity in myeloproliferative disorders. N Engl J of Med 1982; 306: 381–386.
15. Rocca B, Ciabattoni G, Tartaglione R et al. Increased thromboxane biosynthesis in essential thrombocythemia. Tromb Haemost 1995; 74: 1225–1230.
16. Le Blanc K, Lindahl T, Rosendahl K et al. Impaired platelet binding of fibrinogen due to the lower number of GPIIB/IIIA receptors in polycythemia vera. Thrombosis Research 1998; 91: 287–295.
17. Jensen MK, de Nully Brown P, Lund BV et al. Increased platelet activation and abnormality membrane glycoprotein content and redistribution in myeloproliferative disorders. Br J Haematol 2000; 110: 116–124.
18. Michiels JJ, Berneman Z, Schroyens W et al. The paradox of platelet activation and impaired function: platelet‑von Willebrand factor interactions, and the etiology of thrombotic and hemorrhagic manifestations in essential thrombocythemia and polycythemia vera. Semin Tromb Hemost 2006; 32: 589–604.
19. Barbui T, Cortelazzo S, Viero P et al. Thrombohaemorrhagic complications in 101 cases of myeloproliferative disorders: relationship to platelet number and function. Eur J Cancer Clin Oncol 1983; 19: 1593–1599.
20. Fenaux P, Simon M, Caulier MT et al. Clinical course of essential thrombocythemia in 147 cases. Cancer 1990; 66: 549–556.
21. Cesar JM, de Miquel D, Garcia Avello A et al. Platelet dysfunction in primary thrombocythemia using the platelet function analyzer, PFA‑100. Am J Clin Pathol 2005; 123: 772–777.
22. Trelinski J, Tybura M, Chojnowski K. The assessment of platelet function in patients with essential thrombocythemia. Pol Merkur Lekarski 2008; 25: 19–22.
23. 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.
24. Landolfi R, Di Gennaro L, Barbui T et al. Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood 2007; 109: 2446–2552.
25. André‑Kerneïs E, Gaussem P. Role of platelet dysfunction in the haemostatic manifestations occurring during essential thrombocythaemia. Ann Biol Clin 2004; 62: 279–290.
26. Cardin F, Graffeo M, McCormick PA et al. 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.
27. Denninger MH, 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.
28. Anger BR, Seifried E, Scheppach J et al. Budd‑Chiari syndrome and thrombosis of other abdominal vessels in the chronic myeloproliferative diseases. Klinische Wochenschrift 1989; 67: 818–825.
29. Bazzan M, Tamponi G, Schinco P et al. Thrombosis‑free survival and life expectancy in 187 consecutive patients with essential thrombocythemia. Ann Hematol 1999; 78: 539–543.
30. Lengfelder E, Hochhaus A, Kronawitter U et al. Should a platelet limit of 600 × 109/l be used as a diagnostic criterion in essential thrombocythaemia? An analysis of the natural course including early stages. Br J of Haematol 1998; 100: 15–23.
31. Najman Y, Mugnier P, Dresch C et al. Polycythemia vera in young people: an analysis of 58 cases diagnosed before 40 years. Br J Haematol 1987; 67: 285–291.
32. Penka M, Schwarz J, Pavlík T et al: Výsledky léčby nemocných s esenciální trombocytemií a dalšími myeloproliferacemi provázenými trombocytemií – zpráva z registru pacientů léčených Thromboreductinem®. Vnitř Lék 2009; 55: I–XII.
33. Dulíček P, Malý J. Budd‑Chiari syndrom – úloha hematologa v multidisciplinárním přístupu. Vnitř Lék 2008; 54: 842–845.
34. Randi ML, Stucco F, Rossi C et al. Thrombosis and hemorrage in thrombocytosis: evaluation of a large cohort of patients (357 cases). J Med 1991; 22: 213–223.
35. Kessler CM, Klein HG, Havlik RJ. Uncontrolled thrombocytosis in chronic myeloproliferative disorders. Br J Haematol 1982; 50: 157–167.
36. Colombi M, Radaelli F, Zocchi L et al. Thrombotic and hemorrhagic complications in essential thrombocythemia. A retrospective study of 103 patients. Cancer 1991; 67: 2926–2930.
37. Budde U, Scharf RE, Franke P et al. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood 1993; 82: 1749–1757.
38. Budde U, van Genderen PJ. Acquired von Willebrand disease in patients with high platelet counts. Semin Thromb Hemost 1997; 23: 425–431.
39. Federici AB. Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymfoproliferative and myeloproliferative disorders. Semin Hematom 2006; 43 (Suppl 1): S48–S58.
40. Rosendaal FR. Risk factors for venous thrombosis: prevalence, risk, and interaction. Semin Hematol 1997; 34: 171–187.
41. Grupo Italiano Studio Policitemia (GISP), Polycythemia vera: the natural history of 1213 patients followed for 20 years. An Intern Med 1995; 123: 656–664.
42. Barbui T, Finazzi G. Indications for cytoreductive therapy in polycythemia vera and essential thrombocythemia. Hematology 2003; 95: 202–209.
43. Ruggeri M, Gisslinger H, Tosseto A et al. Factor V Leiden mutation carriership and venous thromboembolism in polycythemia vera and essential thrombocythemia. Am J Hemat 2002; 71: 1–6.
44. Landolfi R, Di Gennaro L, Falanga A. Thrombosis in myeloproliferative disorders: pathogenic factor and speculation. Leukemia 2008; 22: 2020–2028.
45. Marchetti M, Castoldi E, Spronk HM et al. Trombin generation and activated protein C resistence in patients with essential thrombocythemia and polycythemia vera. Blood 2008; 1112: 4061–4068.
46. Penka M, Schwarz J, Pytlík R et al. Doporučený postup diagnostiky a terapie esenciální trombocytemie a trombocytemie provázející jiné myeloproliferativní choroby. Vnitř Lék 2005; 51: 555–565.
47. Tefferi A, Mesa RA, Nagorney DM et al. Splenectomy in myelofibrosis with myeloid metaplasma: a single‑institution experience with 223 patients. Blood 2000; 95: 2226–2233.
48. The Seventh ACCP Conference on Antithrombotic and Thrombotic therapy. Chest 2004; 126: 341S.
49. Pearson TC. Hemorheologic considerations in the pathogenesis of vascular occlusive events in polycythemia vera. Semin Thromb Hemost 1997; 23: 433–439.
50. Barbui T, Barosi G, Grossi A et al. Practise guidelines for the therapy of essential thrombocythemia. A statement from the Italian Society of Experimental Hematology. Hematologica 2004; 89: 215–232.
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