Vol 13, No 2 (2022)
Review paper
Published online: 2022-10-20

open access

Page views 738
Article views/downloads 370
Get Citation

Connect on Social Media

Connect on Social Media

Diagnosis and treatment of immune thrombocytopenia in Poland

Piotr Małecki1, Sebastian Grosicki1
Hematology in Clinical Practice 2022;13(2):49-54.

Abstract

Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by an isolated platelet count of less than 100,000/μL caused by destruction and reduced production of platelets. Systemic steroid therapy is a mainstay of first-line treatment; however, additional treatment lines are usually necessary due to the very high recurrence rate after therapy completion and steroid resistance. Standard treatment options for steroid resistance or steroid dependence include splenectomy, rituximab or thrombopoietin receptor agonists.

The article presents current data on the diagnosics and therapy of ITP, considering the limited access to some forms of treatment in Poland.

Article available in PDF format

View PDF Download PDF file

References

  1. Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019; 3(23): 3829–3866.
  2. Matzdorff A, Wörmann B. [Diagnosis and therapy of immune thrombocytopenia]. Dtsch Med Wochenschr. 2018; 143(15): 1076–1081.
  3. Abrahamson PE, Hall SA, Feudjo-Tepie M, et al. The incidence of idiopathic thrombocytopenic purpura among adults: a population-based study and literature review. Eur J Haematol. 2009; 83(2): 83–89.
  4. Schoonen WM, Kucera G, Coalson J, et al. Epidemiology of immune thrombocytopenic purpura in the General Practice Research Database. Br J Haematol. 2009; 145(2): 235–244.
  5. Terrell DR, Beebe LA, Vesely SK, et al. The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports. Am J Hematol. 2010; 85(3): 174–180.
  6. Cines DB, Bussel JB, Liebman HA, et al. The ITP syndrome: pathogenic and clinical diversity. Blood. 2009; 113(26): 6511–6521.
  7. Neunert CE, Buchanan GR, Blanchette V, et al. Relationships among bleeding severity, health-related quality of life, and platelet count in children with immune thrombocytopenic purpura. Pediatr Blood Cancer. 2009; 53(4): 652–654.
  8. Neunert C, Noroozi N, Norman G, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost. 2015; 13(3): 457–464.
  9. Psaila B, Petrovic A, Page LK, et al. Intracranial hemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases. Blood. 2009; 114(23): 4777–4783.
  10. Li S, Molony JT, Cetin K, et al. Rate of bleeding-related episodes in elderly patients with primary immune thrombocytopenia: a retrospective cohort study. Curr Med Res Opin. 2018; 34(2): 209–216.
  11. Bennett CM, Neunert C, Grace RF, et al. Predictors of remission in children with newly diagnosed immune thrombocytopenia: Data from the Intercontinental Cooperative ITP Study Group Registry II participants. Pediatr Blood Cancer. 2018; 65(1).
  12. Schifferli A, Holbro A, Chitlur M, et al. Intercontinental Cooperative ITP Study Group (ICIS). A comparative prospective observational study of children and adults with immune thrombocytopenia: 2-year follow-up. Am J Hematol. 2018; 93(6): 751–759.
  13. Matschke J, Müller-Beissenhirtz H, Novotny J, et al. A randomized trial of daily prednisone versus pulsed dexamethasone in treatment-naïve adult patients with immune thrombocytopenia: EIS 2002 study. Acta Haematol. 2016; 136(2): 101–107.
  14. Zimmer J, Andrès E, Noel E, et al. Current management of adult idiopathic thrombocytopenic purpura in practice: a cohort study of 201 patients from a single center. Clin Lab Haematol. 2004; 26(2): 137–142.
  15. Wei Yu, Ji Xb, Wang Yw, et al. High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial. Blood. 2016; 127(3): 296–302; quiz 370.
  16. Wang Li, Gao Z, Chen XP, et al. Efficacy and safety of thrombopoietin receptor agonists in patients with primary immune thrombocytopenia: a systematic review and meta-analysis. Sci Rep. 2016; 6: 39003.
  17. Kuter D, Bussel J, Lyons R, et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. The Lancet. 2008; 371(9610): 395–403.
  18. Shirasugi Y, Ando K, Miyazaki K, et al. Romiplostim for the treatment of chronic immune thrombocytopenia in adult Japanese patients: a double-blind, randomized Phase III clinical trial. Int J Hematol. 2011; 94(1): 71–80.
  19. Cheng G, Saleh M, Marcher C, et al. Eltrombopag for management of chronic immune thrombocytopenia (RAISE): a 6-month, randomised, phase 3 study. The Lancet. 2011; 377(9763): 393–402.
  20. Bussel J, Provan D, Shamsi T, et al. Effect of eltrombopag on platelet counts and bleeding during treatment of chronic idiopathic thrombocytopenic purpura: a randomised, double-blind, placebo-controlled trial. The Lancet. 2009; 373(9664): 641–648.
  21. Vesely SK, Perdue JJ, Rizvi MA, et al. Management of adult patients with persistent idiopathic thrombocytopenic purpura following splenectomy: a systematic review. Ann Intern Med. 2004; 140(2): 112–120.
  22. Moulis G, Sailler L, Sommet A, et al. Rituximab versus splenectomy in persistent or chronic adult primary immune thrombocytopenia: an adjusted comparison of mortality and morbidity. Am J Hematol. 2014; 89(1): 41–46.
  23. Kojouri K, Vesely SK, Terrell DR, 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): 2623–2634.
  24. Ahmed R, Devasia AJ, Viswabandya A, et al. Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children : Splenectomy in ITP. Ann Hematol. 2016; 95(9): 1429–1434.
  25. Guan Y, Wang S, Xue F, et al. Long-term results of splenectomy in adult chronic immune thrombocytopenia. Eur J Haematol. 2017; 98(3): 235–241.
  26. Ghanima W, Khelif A, Waage A, et al. RITP study group. Rituximab as second-line treatment for adult immune thrombocytopenia (the RITP trial): a multicentre, randomised, double-blind, placebo-controlled trial. The Lancet. 2015; 385(9978): 1653–1661.
  27. Tran H, Brighton T, Grigg A, et al. A multi-centre, single-arm, open-label study evaluating the safety and efficacy of fixed dose rituximab in patients with refractory, relapsed or chronic idiopathic thrombocytopenic purpura (R-ITP1000 study). Br J Haematol. 2014; 167(2): 243–251.
  28. Khellaf M, Charles-Nelson A, Fain O, et al. Safety and efficacy of rituximab in adult immune thrombocytopenia: results from a prospective registry including 248 patients. Blood. 2014; 124(22): 3228–3236.
  29. Bussel JB, Arnold DM, Boxer MA, et al. Long-term fostamatinib treatment of adults with immune thrombocytopenia during the phase 3 clinical trial program. Am J Hematol. 2019; 94(5): 546–553.
  30. Robak T, Kaźmierczak M, Jarque I, et al. Phase 2 multiple-dose study of an FcRn inhibitor, rozanolixizumab, in patients with primary immune thrombocytopenia. Blood Adv. 2020; 4(17): 4136–4146.



Hematology in Clinical Practice