Essential thrombocytemia and other myeloproliferations with thrombocytemia in the data of the register of patients treated with Thromboreductin® till the end of 2006


Authors: M. Penka 1;  J. Schwarz 2;  T. Pavlík 3;  R. Pytlík 4;  M. Doubek 5;  P. Ďulíček 6;  J. Kissová 1;  A. Hluší 7;  M. Schutzova 8;  O. Černá 9;  Y. Brychtová 1;  T. Szotkowski 7;  Z. Volková 2;  J. Seghetová 10;  V. Vozobulová 8;  I. Hadačová 10;  I. Hochová 10;  J. Voglová 6;  T. Lhoťanová 11;  B. Bubeník 12;  M. Vránová 13;  M. Mičaníková 13;  L. Dušek 3
Authors‘ workplace: Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Miroslav Penka, CSc. 1;  Oddělení klinické hematologie FN Motol, Praha, přednostka prim. MUDr. Ivana Hochová 10;  Krevní centrum FNsP Ostrava, přednostka prim. MUDr. Zuzana Čermáková 11;  Krevní centrum s. r. o., Frýdek-Místek, přednosta prim. MUDr. Boris Bubeník 12;  Oddělení klinické hematologie Městské nemocnice Ostrava-Fifejdy, přednostka prim. MUDr. Milena Vránová 13;  Ústav hematologie a krevní transfuze Praha, ředitel prof. MUDr. Pavel Klener, DrSc. 2;  Institut biostatistiky a analýz MU Brno, přednosta doc. RNDr. Ladislav Dušek, CSc. 3;  I. interní klinika 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Pavel Klener, DrSc. 4;  Interní hematoonkologická klinika LF MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc. 5;  Oddělení klinické hematologie II. interní kliniky LF UK a FN Hradec Králové, přednosta prof. MUDr. Jaroslav Malý, CSc. 6;  Hemato-onkologická Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Karel Indrák, DrSc. 7;  Hematoloogicko-onkologické oddělení FN Plzeň, přednosta prim. MUDr. Vladimír Koza 8;  Oddělení klinické hematologie FN Královské Vinohrady Praha, přednosta doc. MUDr. Tomáš Kozák, Ph. D. 9
Published in: Vnitř Lék 2007; 53(6): 653-661
Category: Original Contributions

Overview

Since 2005, registers of patients treated with Thromboreductin® (anagrelid) kept by some centres in the Czech Republic have been supplied with data concerning patients whose treatment with this preparation started in 2004. The purpose of the register is to record responses to therapy by Thromboreductin® and adverse events in patients with essential thrombocytemia and other myeloproliferations, and to subsequently analyse the data. Another objective is to detect predisposition to clinical symptomatology and disease complications. Apart from thrombocyte count, additional risk factors are monitored. The database currently contains data for 336 patients. Initial analyses of data from the register point to the fact that anagrelid is a highly effective thromboreductive agent the administration of which is associated with relatively low incidence of adverse events (11.8 %) of mild and usually transitory nature. The therapeutic objective is attained at a relatively slow rate (according to overall stratification under 400 or under 600 × 109/l thrombocytes), which is probably due to insufficient dose adjustment.

Key words:
anagrelid – essential thrombocytemia – myeloproliferation – register of patients – Thromboreductin®


Sources

1. Abe Andes W, Noveck RJ, Fleming JS. Inhibition of platelet production induced by an antiplatelet drug, anagrelide, in normal volunteers. Thromb Haemost 1984; 52: 325.

2. 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 Italian Group for Bone Marrow Transplantation. Haematologica 2004; 89: 215-232.

3. Brière JB. Essential thrombocythaemia. Orphanet J Rare Dis 2007; 2-3: 1-17.

4. Cortelazzo S, Finazzi G, Ruggeri M et al. Hydoxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med 1995; 332: 113.

5. Doubek M, Brychtová Y, Doubek R et al. Anagrelide in pregnancy: report of a case of essential thrombocythaemia. Ann Oncol 2004; 83: 726-727.

6. Elliott MA, Tefferi A. Interferon-α therapy in Polycythemia Vera and essential Thrombocythemia. Semin Thromb Hemost 1997; 23: 463.

7. Green A, Campbell P, Buck G et al. The Medical Research Council PT1 trial in essential thrombocythaemia. Blood 2004; 104: 5a-6a (Abstr. 6).

8. Harrison CN, Gale RE, Machin SK et al. 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.

9. Jaffe ES, Harris NL, Stein H et al (eds). World Health Organization classification of tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press 2001: 352.

10. Kralovics R, Teo SS, Li S et al. Acquisition of the V617F mutation of JAK2 is a late genetic event in a subset of patients with myeloproliferative disorders. Blood 2006; 108: 1377-1380.

11. Landolfi R, Rocca B, Patrono C. Bleeding and thrombosis in myeloproliferative disorders: mechanism and treatment. Crit Rev Oncol Hematol 1995; 20: 203-222.

12. Messa RA, Tefferi A, Jacobsen SJ et al. Population-based incidence and survival figures in Essential Thrombocythemia and agnogenic myeloid meta-plasia: an Olmsted County study. Am J Hematol 1999; 61: 10-15.

13. Michiels JJ, Kutti J, Stark P et al. Diagnosis, pathogenesis and treatment of the myeloproliferative disorders essential thrombocythemia, polycythemia vera and essential megakaryocytic granulocytic metaplasia and myelofibrosis. Netherlands J Med 1999; 54: 46-62.

14. Michiels JJ, De Raeve H, Hebeda K et al. WHO bone marrow features and European clinical, molecular, and pathological (ECMP) criteria for the diagnosis of myeloproliferative disorders. Leuk Res 2007; In press.

15. 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.

16. Murphy S, Peterson P, Iland H et al. Experience of the Polycythemia Vera Study Group with essential thrombocythemia: a final report on diagnostic criteria, survival, and leukemic transition by treatment. Semin Hematol 1997; 34: 29-39.

17. Penka M, Schwarz J, Pytlík R et al. Doporučený postup diagnostiky a terapie esenciální trombocytemie a trombocytemie provázející myeloproliferativní onemocnění. Vnitř Lék 2005; 51: 741-751.

18. Penka M, Doubek M, Schwarz J et al. Anagrelid v léčbě esenciální trombocytemie a dalších myeloproliferací s trombocytemií sledovaných v registru pacientů ČR. Vnitř Lék 2006; 52: 498-503.

19. Petrides PE Anagrelid: decade of clinical experiences with its use for the treatment of primary thrombocythaemia. Expert Opin Pharmacother 2004; 5: 1781-1798.

20. Petrides PE, Beykirch MK, Trapp OM. Anagrelide, a novel platelet lowering option in essential thrombocythaemia: treatment experiences in 48 patients in Germany. Eur J Haematol 1998; 61: 71.

21. Petitt RM, Silverstein MN, Petrone ME. Anagrelide for control of thrombocythemia in polycythemia and other myeloproliferative disorders. Semin Hematol 1997; 34: 51.

22. Pytlík R, Cmunt E, Kleibl Z et al. Úloha anagrelidu v léčbě esenciální trombocytemie. Transfuz Hematol Dnes 2004; 10: 154-160.

23. Schwarz J, Pytlík R, Doubek M et al. Analysis of risk factors: the rationale of the guidelines for diagnosis and treatment of Ph-negative myeloproliferative disorders with thrombocythemia of the Czech Hematological Society. Sem Thromb Haemost 2006; 32: 231-245.

24. Schwarz J, Markova J, Volkova Z et al. The majority of patients with Ph-negative MPD with thrombocythemia and major thrombosis have either inherited thrombophila or JAK2 V617F gene mutation. Abstr. Second ESH Euroconference on Myeloproliferative Disorders: Molecular Pathogenesis and Therapy, Madeira, poster 23, 2006.

25. Silverstein MN, Tefferi A. Treatment of essential thrombocythemia with anagrelide. Semin Hematol 1999; 36: 23-25.

26. Solberg LA jr, Oles KJ, Tarach J et al. The effects of anagrelid on human megakaryocytopoiesis. Blood 1989; 6274 (Suppl 1): 20a.

27. Steurer M, Gastl G, Jedrzejczak W et al. Anagrelide for thrombocytosis in myeloproliferative disorders: a prospective study to assess efficacy and adverse event profile. Cancer 2004; 101: 2239-2246.

28. Storen EC, Tefferi A Long-term use of anagrelid in young patients with essential thrombocythemia. Blood 2001; 97: 836-866.

29. Thiele J, Kvasnicka HM, Graeff AS et al. Follow-up examinations including sequential bone marrow biopsies in essential thrombocythemia (ET): A retrospective clinicopathological study of 120 patients. Am J Hematol 2002; 70: 283-291.

30. Thiele J, Kvasnicka HM, Zankovich R et al. Clinical and morphological criteria for the diagnosis of prefibrotic idiopathic (primary) myelofibrosis. Ann Hematol 2001; 80: 160-165.

31. Tsimberidou MA, Colburn DE, Welch MA et al. Anagrelide and imatinib mesylate combination therapy in patients with chronic myeloproliferative disorders. Cancer Chemother Pharmacol 2003; 52: 229-234.

32. Wright CA, Tefferi A. A single institutional experience with 43 pregnancies in essential thrombocythemia. Eur J Haematol 2001; 66: 152.

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