Иммунная тромбоцитопения: клинические проявления и ответ на терапию. Промежуточный анализ данных Российского регистра пациентов с первичной иммунной тромбоцитопенией и обзор литературы

Первичная иммунная тромбоцитопения (ИТП) относится к редким (орфанным) заболеваниям. Наиболее частыми проявлениями ИТП являются пурпура, петехии и кровотечения, при этом многие пациенты не имеют клинических симптомов заболевания или отмечают минимальные проявления кровоточивости. В последние годы были опубликованы современные международные рекомендации по лечению ИТП, при этом подход к лечению каждого конкретного пациента всегда определяется на основе индивидуальной оценки течения заболевания. В данной статье представлены результаты промежуточного анализа особенностей клинического течения и терапевтических подходов к ИТП по данным Российского регистра пациентов с иммунной тромбоцитопенией, а также обзор данных литературы по лечению ИТП.

1. Abrahamson P.E., Hall S.A., Feudjo-Tepie M. et al. The incidence of idiopathic thrombocytopenic purpura (ITP) among adults: a population-based study and literature review. Eur J Haematol 2009;83:83–9.

2. Aoki T., Harada Y., Matsubara E. et al. Thrombopoietin receptor agonists in refractory immune thrombocytopenia: differential responses to eltrombopag and romiplostim: a case report and possible explanations. J Clin Pharm Ther 2012 Dec;37(6):729–32.

3. Arnold D.M., Dentali F., Crowther M.A. et al. Systematic review: efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Ann Intern Med 2007;146(1):25–33.

4. Arnold D.M., Heddle N.M., Carruthers J. et al. A pilot randomized trial of adjuvant rituximab or placebo for non-splenectomized patients with immune thrombocytopenia. Blood 2012 Feb 9;119(6):1356–62.

5. Bizzoni L., Mazzucconi M., Gentile M. et al. Idiopathic thrombocytopenic purpura (ITP) in the eldery: clinical course in 178 patients. Eur J Haematol 2006;76(3):210–6. 6. Boccia R., Kuter D.J., Rummel M.J. et al. The effects of romiplostim or standard of care (SOC) on splenectomy and treatment failure of patients who had immune thrombocytopenia (ITP) for less than or equal to one year. Blood 2010;116(21):3702.

6. Bussel J.B., Kuter D.J., Pullarkat V. et al. Safety and efficacy of long-term treatment with romiplostim in thrombocytopenic patients with chronic ITP. Blood 2009;113:2161–71.

7. Bussel J., Rodeghiero F., Lyons R.M. et al. Sustained hemostatic platelet counts in adults with immune thrombocytopenia (ITP) following cessation of treatment with the TPO receptor agonist romiplostim: report of 9 cases. Blood 2011;118(21):3281.

8. Chen J., Herceg-Harjacek L., Groopman J., Grabarek J. Regulation of platelet activation in vitro by the c-Mpl ligand, thrombopoietin. Blood1995;86:4054–62.

9. Cheng G., Saleh M.N., Marcher C. et al. Eltrombopag for management of chronic immune thrombocytopenia (RAISE): a 6-month, randomised, phase 3 study. Lancet 2011;377:393–402.

10. Cooper K.L., Fitzgerald P., Dillingham K. et al. Romiplostim and eltrombopag for immune thrombocytopenia: methods for indirect comparison. Int J Technol Assess Health Care 2012;28(3):

11. –58.

12. Cooper N., Stasi R., Cunningham- Rundles S. et al. The efficacy and safety of B-cell depletion with anti-CD20 monoclonal antibody in adults with chronic immune thrombocytopenic purpura. Br J Haematol 2004;125(2):232–9.

13. Fogarty P.F. Chronic immune thrombocytopenia in adults: epidemiology and clinical presentation. Hematol Oncol Clin N Am 2009;23:1213–21.

14. Frederiksen H., Schmidt K. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood 1999;94(3):909–13.

15. Godeau B., Porcher R., Fain O. et al. Rituximab efficacy and safety in adults splenectomy candidates with chronic immune thrombocytopenic purpura: results of a prospective multicenter phase 2 study. Blood 2008;112(4):999–1004.

16. Jenssens A., Tarantino M., Bird R. et al. Final results from an international, multi-center, single-arm study evaluating the safety and efficacy of romiplostim in adults with primary immune thrombocytopenia (ITP). Blood 2011;118(21):3279.

17. Kojouri K., Vesely S.K., Terrell D.R. et al. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response and surgical complications Blood 2004;104(9):2623–34.

18. Kuter D.J., Bussel J.B., Lyons R.M. et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomized controlledtrial. Lancet 2008;371(9610):395–403.

19. Kuter D., Rummel M., Boccia R. et al. Romiplostim or standart of care in patients with immune thrombocytopenia. N Engl J Med 2010;363(20):1889–99.

20. McMillan R., Durette C. Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood 2004;104(4):956–60.

21. Neylon A.J., Saunders P.W., Haward M.R. et al. Clinically significant newly presenting autoimmune

22. thrombocytopenic purpura in adults: a prospective study of population-based cohort of 245 patients. Br J Haematol 2003;122(6):966–74.

23. Patel V.L., Mahevas M., Lee S. Y. et al. Outcomes 5 years after response to rituximab therapy in children and adults with mmune thrombocytopenia.Blood 2012;119:5989–95.

24. Portielje J.E., Westendorp R.G., Kluin- Nelemans H.C., Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001;97(9):2549–54.

25. Rodeghiero F., Stasi R., Gernsheimer T. et al. Standartization of terminology definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international woring group. Blood 2009;113(11):2386–93.

26. Vianelly N., Galli M., de Vivo A. et al. Gruppo Italiano per lo Studio delle Malattie Ematologiche dell`Adulto. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases. Haematologica 2005; 90(1):72–7.

27. Wasser J., Boccia R., Lyons R.M. Use of rituximab in a study comparing the thrombopoietin mimetic romiplostim with standard of care (SOC) in a patients with immune thrombocytopenia (ITP). Blood 2011;118(21):3282.

28. Wong G.C., Lee L.H. A study of idiopathic thrombocytopenic purpura (ITP) patients over a ten-year period. Ann Acad Med Singapore 1998;27(6):789–93.