Blood Res.  2017 Dec;52(4):254-263. 10.5045/br.2017.52.4.254.

Management of immune thrombocytopenia: Korean experts recommendation in 2017

• Jang JH
• Kim JY
• Mun YC
• Bang SM
• Lim YJ
• Shin DY
• Choi YB
• Yhim HY
• Lee JW
• Kook H
• on the behalf of Korean Aplastic Anemia Working Party

• KMID: 2405061
• DOI: http://doi.org/10.5045/br.2017.52.4.254

Abstract

Management options for patients with immune thrombocytopenia (ITP) have evolved substantially over the past decades. The American Society of Hematology published a treatment guideline for clinicians referring to the management of ITP in 2011. This evidence-based practice guideline for ITP enables the appropriate treatment of a larger proportion of patients and the maintenance of normal platelet counts. Korean authority operates a unified mandatory national health insurance system. Even though we have a uniform standard guideline enforced by insurance reimbursement, there are several unsolved issues in real practice in ITP treatment. To optimize the management of Korean ITP patients, the Korean Society of Hematology Aplastic Anemia Working Party (KSHAAWP) reviewed the consensus and the Korean data on the clinical practices of ITP therapy. Here, we report a Korean expert recommendation guide for the management of ITP.

Keyword

Aplastic Anemia Working Party; ITP; Recommendation; Management

MeSH Terms

Anemia, Aplastic
Clothing
Consensus
Evidence-Based Practice
Hematology
Humans
Insurance
National Health Programs
Platelet Count
Purpura, Thrombocytopenic, Idiopathic*

Reference

1. Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med. 2002; 346:995–1008.

2. Barsam SJ, Psaila B, Forestier M, et al. Platelet production and platelet destruction: assessing mechanisms of treatment effect in immune thrombocytopenia. Blood. 2011; 117:5723–5732.

3. Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011; 117:4190–4207.

4. Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009; 113:2386–2393.

5. Stasi R, Amadori S, Osborn J, Newland AC, Provan D. Long-term outcome of otherwise healthy individuals with incidentally discovered borderline thrombocytopenia. PLoS Med. 2006; 3:e24.

6. Lambert MP, Gernsheimer TB. Clinical updates in adult immune thrombocytopenia. Blood. 2017; 129:2829–2835.

7. Cooper N. A review of the management of childhood immune thrombocytopenia: how can we provide an evidence-based approach? Br J Haematol. 2014; 165:756–767.

8. Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010; 115:168–186.

9. Stasi R, Sarpatwari A, Segal JB, et al. Effects of eradication of Helicobacter pylori infection in patients with immune thrombocytopenic purpura: a systematic review. Blood. 2009; 113:1231–1240.

10. Shin WG, Lee SW, Baik GH, et al. Eradication Rates of Helicobacter pylori in Korea Over the Past 10 years and Correlation of the Amount of Antibiotics Use: Nationwide Survey. Helicobacter. 2016; 21:266–278.

11. Kim H, Lee WS, Lee KH, et al. Efficacy of Helicobacter pylori eradication for the 1st line treatment of immune thrombocytopenia patients with moderate thrombocytopenia. Ann Hematol. 2015; 94:739–746.

12. McMillan R, Wang L, Tani P. Prospective evaluation of the immunobead assay for the diagnosis of adult chronic immune thrombocytopenic purpura (ITP). J Thromb Haemost. 2003; 1:485–491.

13. Diz-Küçükkaya R, Hacihanefioğlu A, Yenerel M, et al. Antiphospholipid antibodies and antiphospholipid syndrome in patients presenting with immune thrombocytopenic purpura: a prospective cohort study. Blood. 2001; 98:1760–1764.

14. Davoren A, Bussel J, Curtis BR, Moghaddam M, Aster RH, McFarland JG. Prospective evaluation of a new platelet glycoprotein (GP)-specific assay (PakAuto) in the diagnosis of autoimmune thrombocytopenia (AITP). Am J Hematol. 2005; 78:193–197.

15. Cines DB, Bussel JB. How I treat idiopathic thrombocytopenic purpura (ITP). Blood. 2005; 106:2244–2251.

16. Chong BH, Brighton TA, Baker RI, Thurlow P, Lee CH;. Once-daily enoxaparin in the outpatient setting versus unfractionated heparin in hospital for the treatment of symptomatic deep-vein thrombosis. J Thromb Thrombolysis. 2005; 19:173–181.

17. Perricone C, Ceccarelli F, Nesher G, et al. Immune thrombocytopenic purpura (ITP) associated with vaccinations: a review of reported cases. Immunol Res. 2014; 60:226–235.

18. Elalfy MS, Nugent D. Viruses, anti-viral therapy, and viral vaccines in children with immune thrombocytopenia. Semin Hematol. 2016; 53:Suppl 1. S70–S72.

19. Cohen YC, Djulbegovic B, Shamai-Lubovitz O, Mozes B. The bleeding risk and natural history of idiopathic thrombocytopenic purpura in patients with persistent low platelet counts. Arch Intern Med. 2000; 160:1630–1638.

20. Portielje JE, Westendorp RG, Kluin-Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001; 97:2549–2554.

21. Arnold DM. Platelet count or bleeding as the outcome in ITP trials? Am J Hematol. 2012; 87:945–946.

22. Michel M, Rauzy OB, Thoraval FR, et al. Characteristics and outcome of immune thrombocytopenia in elderly: results from a single center case-controlled study. Am J Hematol. 2011; 86:980–984.

23. Cheng Y, Wong RS, Soo YO, et al. Initial treatment of immune thrombocytopenic purpura with high-dose dexamethasone. N Engl J Med. 2003; 349:831–836.

24. Din B, Wang X, Shi Y, Li Y. Long-term effect of high-dose dexamethasone with or without low-dose dexamethasone maintenance in untreated immune thrombocytopenia. Acta Haematol. 2015; 133:124–128.

25. Godeau B, Chevret S, Varet B, et al. Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial. Lancet. 2002; 359:23–29.

26. Mazzucconi MG, Fazi P, Bernasconi S, et al. HD-DXM) in previously untreated patients affected by idiopathic thrombocytopenic purpura: a GIMEMA experience. Blood. 2007; 109:1401–1407.

27. Wei Y, Ji XB, Wang YW, et al. High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial. Blood. 2016; 127:296–302.

28. Zaja F, Baccarani M, Mazza P, et al. Dexamethasone plus rituximab yields higher sustained response rates than dexamethasone monotherapy in adults with primary immune thrombocytopenia. Blood. 2010; 115:2755–2762.

29. Godeau B, Lesage S, Divine M, Wirquin V, Farcet JP, Bierling P. Treatment of adult chronic autoimmune thrombocytopenic purpura with repeated high-dose intravenous immunoglobulin. Blood. 1993; 82:1415–1421.

30. Schiavotto C, Ruggeri M, Rodeghiero F. Adverse reactions after high-dose intravenous immunoglobulin: incidence in 83 patients treated for idiopathic thrombocytopenic purpura (ITP) and review of the literature. Haematologica. 1993; 78:Suppl 2. 35–40.

31. Gaines AR. Disseminated intravascular coagulation associated with acute hemoglobinemia or hemoglobinuria following Rh(0)(D) immune globulin intravenous administration for immune thrombocytopenic purpura. Blood. 2005; 106:1532–1537.

32. Palandri F, Polverelli N, Sollazzo D, et al. Have splenectomy rate and main outcomes of ITP changed after the introduction of new treatments? A monocentric study in the outpatient setting during 35 years. Am J Hematol. 2016; 91:E267–E272.

33. Kumar S, Diehn FE, Gertz MA, Tefferi A. Splenectomy for immune thrombocytopenic purpura: long-term results and treatment of postsplenectomy relapses. Ann Hematol. 2002; 81:312–319.

34. 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; 95:1429–1434.

35. Thai LH, Mahévas M, Roudot-Thoraval F, et al. Long-term complications of splenectomy in adult immune thrombocytopenia. Medicine (Baltimore). 2016; 95:e5098.

36. Guan Y, Wang S, Xue F, et al. Long-term results of splenectomy in adult chronic immune thrombocytopenia. Eur J Haematol. 2017; 98:235–241.

37. Cuker A, Cines DB, Neunert CE. Controversies in the treatment of immune thrombocytopenia. Curr Opin Hematol. 2016; 23:479–485.

38. Ghanima W, Khelif A, Waage A, et al. Rituximab as second-line treatment for adult immune thrombocytopenia (the RITP trial): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet. 2015; 385:1653–1661.

39. George JN, Woolf SH, Raskob GE, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood. 1996; 88:3–40.

40. Beck CE, Nathan PC, Parkin PC, Blanchette VS, Macarthur C. Corticosteroids versus intravenous immune globulin for the treatment of acute immune thrombocytopenic purpura in children: a systematic review and meta-analysis of randomized controlled trials. J Pediatr. 2005; 147:521–527.

41. Stasi R. Immune thrombocytopenia: pathophysiologic and clinical update. Semin Thromb Hemost. 2012; 38:454–462.

42. Jung JY, O AR, Kim JK, Park M. Clinical course and prognostic factors of childhood immune thrombocytopenia: single center experience of 10 years. Korean J Pediatr. 2016; 59:335–340.

43. Choi HS, Ji MH, Kim SJ, Ahn HS. Platelet count recovery after intravenous immunoglobulin predicts a favorable outcome in children with immune thrombocytopenia. Blood Res. 2016; 51:95–101.

44. Labrosse R, Vincent M, Nguyen UP, Chartrand C, Di Liddo L, Pastore Y. Using a standardised protocol was effective in reducing hospitalisation and treatment use in children with newly diagnosed immune thrombocytopenia. Acta Paediatr. 2017; 106:1617–1623.

45. Shahgholi E, Vosough P, Sotoudeh K, et al. Intravenous immune globulin versus intravenous anti-D immune globulin for the treatment of acute immune thrombocytopenic purpura. Indian J Pediatr. 2008; 75:1231–1235.

46. Tarantino MD, Young G, Bertolone SJ, et al. Single dose of anti-D immune globulin at 75 microg/kg is as effective as intravenous immune globulin at rapidly raising the platelet count in newly diagnosed immune thrombocytopenic purpura in children. J Pediatr. 2006; 148:489–494.

47. Son DW, Jeon IS, Yang SW, Cho SH. A single dose of anti-D immunoglobulin raises platelet count as efficiently as intravenous immunoglobulin in newly diagnosed immune thrombocytopenic purpura in Korean children. J Pediatr Hematol Oncol. 2008; 30:598–601.

48. Liang Y, Zhang L, Gao J, Hu D, Ai Y. Rituximab for children with immune thrombocytopenia: a systematic review. PLoS One. 2012; 7:e36698.

49. Cooper N, Bussel JB. The long-term impact of rituximab for childhood immune thrombocytopenia. Curr Rheumatol Rep. 2010; 12:94–100.

50. Yri OE, Torfoss D, Hungnes O, et al. Rituximab blocks protective serologic response to influenza A (H1N1) 2009 vaccination in lymphoma patients during or within 6 months after treatment. Blood. 2011; 118:6769–6771.

51. Wali YA, Al Lamki Z, Shah W, Zacharia M, Hassan A. Pulsed high-dose dexamethasone therapy in children with chronic idiopathic thrombocytopenic purpura. Pediatr Hematol Oncol. 2002; 19:329–335.

52. Kühne T, Freedman J, Semple JW, Doyle J, Butchart S, Blanchette VS. Platelet and immune responses to oral cyclic dexamethasone therapy in childhood chronic immune thrombocytopenic purpura. J Pediatr. 1997; 130:17–24.

53. Borgna-Pignatti C, Rugolotto S, Nobili B, et al. A trial of high-dose dexamethasone therapy for chronic idiopathic thrombocytopenic purpura in childhood. J Pediatr. 1997; 130:13–16.

54. Adams DM, Kinney TR, O'Branski-Rupp E, Ware RE. High-dose oral dexamethasone therapy for chronic childhood idiopathic thrombocytopenic purpura. J Pediatr. 1996; 128:281–283.

55. Feng S, Qiu Y, Li X, et al. Laparoscopic versus open splenectomy in children: a systematic review and meta-analysis. Pediatr Surg Int. 2016; 32:253–259.

56. Mantadakis E, Buchanan GR. Elective splenectomy in children with idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol. 2000; 22:148–153.

57. Aronis S, Platokouki H, Avgeri M, Pergantou H, Keramidas D. Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children. Acta Paediatr. 2004; 93:638–642.

58. Li J, Yang C, Xia Y, et al. Thrombocytopenia caused by the development of antibodies to thrombopoietin. Blood. 2001; 98:3241–3248.

59. Cuker A, Neunert CE. How I treat refractory immune thrombocytopenia. Blood. 2016; 128:1547–1554.

60. Bussel JB, de Miguel PG, Despotovic JM, et al. Eltrombopag for the treatment of children with persistent and chronic immune thrombocytopenia (PETIT): a randomised, multicentre, placebo-controlled study. Lancet Haematol. 2015; 2:e315–e325.

61. Grainger JD, Locatelli F, Chotsampancharoen T, et al. Eltrombopag for children with chronic immune thrombocytopenia (PETIT2): a randomised, multicentre, placebo-controlled trial. Lancet. 2015; 386:1649–1658.

62. Tarantino MD, Bussel JB, Blanchette VS, et al. Romiplostim in children with immune thrombocytopenia: a phase 3, randomised, double-blind, placebo-controlled study. Lancet. 2016; 388:45–54.

63. Cines DB, Gernsheimer T, Wasser J, et al. Integrated analysis of long-term safety in patients with chronic immune thrombocytopaenia (ITP) treated with the thrombopoietin (TPO) receptor agonist romiplostim. Int J Hematol. 2015; 102:259–270.

64. Brynes RK, Orazi A, Theodore D, et al. Evaluation of bone marrow reticulin in patients with chronic immune thrombocytopenia treated with eltrombopag: Data from the EXTEND study. Am J Hematol. 2015; 90:598–601.

65. Yim JY, Kim N, Choi SH, et al. Seroprevalence of Helicobacter pylori in South Korea. Helicobacter. 2007; 12:333–340.

66. Salama A, Kiesewetter H, Kalus U, Movassaghi K, Meyer O. Massive platelet transfusion is a rapidly effective emergency treatment in patients with refractory autoimmune thrombocytopenia. Thromb Haemost. 2008; 100:762–765.

67. Boruchov DM, Gururangan S, Driscoll MC, Bussel JB. Multiagent induction and maintenance therapy for patients with refractory immune thrombocytopenic purpura (ITP). Blood. 2007; 110:3526–3531.

68. Kalmadi S, Tiu R, Lowe C, Jin T, Kalaycio M. Epsilon aminocaproic acid reduces transfusion requirements in patients with thrombocytopenic hemorrhage. Cancer. 2006; 107:136–140.