Management of Adult Immune Thrombocytopenia: Review Article

Abstract

Immune thrombocytopenia (ITP) is an acquired autoimmune disorder defined by isolated thrombocytopenia and the exclusion of other causes of thrombocytopenia.Although the underlying pathophysiology of ITP has been known for more than five decades, therapy has remained empirical. ITP guidelines were published by the American Society of Hematology 15 years ago (updated in 2011) and the British Committee for Standards in Hematology 8 years ago. The primary treatment goal is to prevent severe bleeding rather than achieve normal platelet counts. Nowadays, new therapeutic agents have changed strategies for ITP treatment. In this review,we discuss criteria for treatment, the role of splenectomy and other treatment options along with their side effects, and the treatment of ITP during pregnancy. Key words: Primary; Immune; Thrombocytopenia; Treatment; ITP.

Keywords

• Primary; Immune; Thrombocytopenia; Treatment; ITP.

Erişkin İmmun Trombositopeni ve Tedavisi: Derleme

Öz

İmmün trombositopeni (İTP) diğer trombositopeni yapan nedenlerin olmaması ve izole trombositopeni ile seyreden edinilmiş otoimmün hastalıktır. İTP'nın patofizyolojisi 50 yıldan daha uzun süredir bilinmesine rağmen tedavi empirik olarak kalmıştır. İTP kılavuzu 15 yıl önce Amerikan Hematoloji Derneği(2011 de güncellenmiştir) ve 8 yıl önce de Hematoloji Standartları İngiliz Komitesi tarafından yayınlanmıştır. Tedavinin başlıca amacı normal platelet sayısına ulaşıncaya kadar ciddi kanamayı engellemektir. Günümüzde İTP'de yeni ilaçlarla tedavi stratejileri değişti. Bu derlemede erişkin İTP'de tedavi kriterlerini, tedavi yaklaşımlarını, splenektominin rolünü, yan etkileri ile birlikte diğer tedavi seçeneklerini ve gebelikteki İTP'nın tedavisini tartıştık. Anahtar kelimeler: Primer; Immün; Trombositopeni; Tedavi; İTP.

Anahtar Kelimeler

• Primer; Immün; Trombositopeni; Tedavi; İTP.

References

1. Neunert C, Lim W, Crowther M, et. al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 2011; 21; 117(16): 4190-207.
2. Yang R, Han ZC. Pathogenesis and management of chronic idiopathic thrombocytopenic purpura: an update. Int J Hematol 2000;71:18-24.
3. 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.
4. British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol 2003; 120: 574-596.
5. Provan D, Stasi R, Newland AC, et. al. International consensus report on the investigation and management of primaryimmune thrombocytopenia Blood 2010 14; 115(2): 168-86.
6. Stasi R, Evangelista ML, Stipa E, Buccisano F, Venditti A, Amadori S. Idiopathic thrombocytopenic purpura: current concepts in pathophysiology and management. Thromb Haemost 2008; 99: 4-13.
7. Psaila B, Bussel JB. Immune thrombocytopenic purpura. Hematol Oncol Clin North Am 2007; 21:743-59.
8. Cines DB, McMillan R. Pathogenesis of chronic immune thrombocytopenic purpura. Curr Opin Hematol 2007; 14: 511-4.

9. Provan D, Newland A. Idiopathic thrombocytopenic purpura in adults. J Pediatr Hematol Oncol 2003; 25: 1:4-8.
10. Cines DB, Bussel JB. How I treat idiopathic thrombocytopenic purpura (ITP). Blood 2005; 1; 106: 2244-51.
11. Kaya E, Erkurt MA, Aydogdu I, et. al Retrospective analysis of patients with idiopathic thrombocytopenic purpura from Eastern Anatolia. Med Princ Pract 2007; 16: 100-6.
12. Portielje JE, Westendorp RG, Kluin-Nelemans HC, et. al. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001; 1;97: 2549-54.
13. Godeau B, Provan D, Bussel J. Immune thrombocytopenic purpura in adults. Curr Opin Hematol 2007;14:535-56.
14. Rodeghiero F. First-line therapies for immune thrombocytopenic purpura: re-evaluating the need to treat. Eur J Haematol Suppl 2008; 69: 19-26.
15. Boruchov DM, Gururangan S, Driscoll MC, et. al. Multiagent induction and maintenance therapy for patients with refractory immune thrombocytopenic purpura (ITP). Blood 2007; 110: 3526-31.
16. Kuku I, Aydogdu I, Kaya E, et. al. The early and long-term results of oral high-dose methylprednisolone treatment in adult patients with idiopathic thrombocytopenic purpura. Eur J Haematol 2005; 74: 271-2.
17. Mazzucconi MG, Fazi P, Bernasconi S, et. al. Therapy with high- dose dexamethasone (HDDXM) in previously untreated patients affected by idiopathic thrombocytopenic purpura: a GIMEMA experience. Blood 2007; 109: 1401-7.
18. Stevens W, Koene H, Zwaginga JJ, et. al. Chronic idiopathic thrombocytopenic purpura: present strategy, guidelines and new insights. Neth J Med 2006; 64: 356-63.
19. Ismet A, Irfan K, Emin K, et. al. Splenectomy results in patients with idiopathic thrombocytopenic purpura: 10 years of experience in Turgut Ozal Medical Center. Clin Lab Haem 2004; 26: 211-4.
20. Pace DE, Chiasson PM, Schlachta CM, et. al. Laparoscopic splenectomy for idiopathic thrombocytopenic purpura (ITP). Surg Endosc 2003; 17: 95-8.
21. Cordera F, Long KH, Nagorney DM et. al. Open versus laparoscopic splenectomy for idiopathic thrombocytopenic purpura: clinical and economic analysis. Surgery 2003; 134: 45-52.
22. Lorton JE. Management of asplenic patients. Br J Haematol 1993; 84: 566-9.
23. George JN. Management of patients with refractory immune thrombocytopenic purpura. J Thromb Haemost 2006; 4: 1664- 72.
24. Zhou Z, Yang R. Rituximab treatment for chronic refractory idiopathic thrombocytopenic purpura. Crit Rev Oncol Hematol 2008; 65: 21-31.
25. Psaila B, Bussel JB. Refractory immune thrombocytopenic purpura: current strategies for investigation and management. Br J Haematol 2008; 143: 16-26.
26. Arnold DM, Dentali F, Crowther MA, et. al. Systematic review: efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Ann Intern Med 2007; 21; 147:281.
27. 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; 8;115(14): 2755-62.
28. Stasi R, Evangelista ML, Amadori S. Novel thrombopoietic agents: a review of their use in idiopathic thrombocytopenic purpura. Drugs 2008; 68(7): 901-12.
29. Kuter DJ, Bussel JB, Lyons RM, et. al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. Lancet 2008; 371(9610):395-403.
30. Garvey B. Management of chronic autoimmune thrombocytopenic purpura (ITP) in adults. Transfus Sci 1998; 19: 269-77.
31. Emilia G, Morselli M, Luppi M, et. al. Long-term salvage therapy with cyclosporin A in refractory idiopathic thrombocytopenic purpura. Blood 2002; 99: 1482-5.
32. Kotb R, Pinganaud C, Trichet C, et. al. Efficacy of mycophenolate mofetil in adult refractory auto-immune cytopenias: a single center preliminary study. Eur J Haematol 2005; 75:60-64.
33. Provan D, Moss AJ, Newland AC, et. al.. Efficacy of mycophenolate mofetil as single-agent therapy for refractory immune thrombocytopenic purpura. Am J Hematol 2006; 81:19- 25.
34. Howard J, Hoffbrand AV, Prentice HG, et. al. Mycophenolate mofetil for the treatment of refractory auto-immune haemolytic anemia and auto-immune thrombocytopenia purpura. Br J Haematol 2002; 117: 712-15.
35. 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: 112-120.
36. Damodar S, Viswabandya A, George B, et. al Dapsone for chronic idiopathic thrombocytopenic purpura in children and adults: a report on 90 patients. Eur J Haematol 2005; 75: 328- 331.
37. Godeau B, Durand JM, Roudot-Thoraval F, et. al. Dapsone for chronic autoimmune thrombocytopenic purpura: a report of 66 cases. Br J Haematol 1997; 97: 336-9.
38. Manoharan A. Treatment of refractory idiopathic thrombocytopenic purpura in adults. Br J Haematol 1991; 79: 143-7.
39. Berchtold P, McMillan R. Therapy of chronic idiopathic thrombocytopenic purpura in adults. Blood 1989; 74: 2309-17.
40. Cines DB, McMillan R. Management of adult idiopathic thrombocytopenic purpura. Annu Rev Med 2005; 56: 425-42.
41. Dubbeld P, Hillen HFP, Schouten HC. Interferon treatment of refractory idiopathic thrombocytopenic purpura (ITP). Eur J Haematol 1994; 52: 233-5.
42. Stasi R, Provan D. Management of immune thrombocytopenic purpura in adults. Mayo Clin Proc 2004; 79: 456-7.
43. Emilia G, Luppi M, Torelli G. Infectious agents and human immune diseases: lessons from Helicobacter pylori. Am J Med 2005; 118: 420-1.
44. Emilia G, Luppi M, Zucchini P, et. al. Helicobacter pylori infection and chronic immune thrombocytopenic purpura: long- term results of bacterium eradication and association with bacterium virulence profiles. Blood 2007; 110: 3833-41.
45. Kohda K, Kuga T, Kogawa K, et. al. Effect of Helicobacter pylori eradication on platelet recovery in Japanese patients with chronic idiopathic thrombocytopenic purpura and secondary autoimmune thrombocytopenic purpura. Br J Haematol 2002; 118: 584-8.
46. Michel M, Cooper N, Jean C, et. al. Does Helicobacter pylori initiate or perpetuate immune thrombocytopenic purpura? Blood 2003;103: 890-96.
47. Gernsheimer T, McCrae KR. Immune thrombocytopenic purpura in pregnancy. Curr Opin Hematol 2007; 14: 574-80.
48. Webert KE, Mittal R, Sigouin C et. al. A retrospective 11-year analysis of obstetric patients with idiopathic thrombocytopenic purpura. Blood 2003; 102: 4306-11.
49. Corresponding Author: Mehmet Ali ERKURT
50. Inonu University Faculty of Medicine,
51. Department of Hematology, TR-44069 Malatya
52. Phone: [phone], ext. 4203
53. celluler phone :[phone]
54. Fax: [phone]
55. e-mail: [e-mail]