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ITP in Adults

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Autoimmune Thrombocytopenia

Abstract

Most cases of adult ITP are chronic form, and the onset of the disease is usually insidious. Signs and symptoms vary widely in each case from no hemorrhagic symptom to severe life-threatening bleeding, such as intracranial hemorrhage. In addition to hemorrhagic symptom, treatment-related complications, such as infection and cardiovascular diseases, may lead to poor quality of life of ITP patients. Increase of thrombosis in ITP patients is also demonstrated in epidemiologic studies. Once diagnosed with ITP, screening and eradication of H. pylori should be performed in the regions of its high prevalence like Japan. After exclusion of H. pylori-associated ITP, treatment of ITP should be initiated to maintain safe platelet counts as a goal. First-line treatment is corticosteroids and second-line is splenectomy. For refractory ITP patients, thrombopoietin receptor agonists are highly recommended. Rituximab may be another option to get sustained response.

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References

  1. Liel MS, Michae R, Calverley DC. Thrombocytopenia caused by immunologic platelet destruction. In: Greer JP, Arber DA, Glader B, List AF, Means Jr RT, Paraskevas F, Rodgers GM, editors. Wintrobe’s clinical hematology. 13th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2014. p. 1061–76.

    Google Scholar 

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

    CAS  PubMed  Google Scholar 

  3. Neylon AJ, Saunders PW, Howard MR, Proctor SJ, Taylor PR. Clinically significant newly presenting autoimmune thrombocytopenic purpura in adults: a prospective study of a population-based cohort of 245 patients. Br J Haematol. 2003;122(6):966–74.

    Article  PubMed  Google Scholar 

  4. Stasi R, Stipa E, Masi M, Cecconi M, Scimò MT, Oliva F, et al. Long-term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med. 1995b;98(5):436–42.

    Article  CAS  PubMed  Google Scholar 

  5. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, 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(11):2386–93.

    Article  CAS  PubMed  Google Scholar 

  6. Portielje JE, Westendorp RG, Kluin-Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001;97(9):2549–54.

    Article  CAS  PubMed  Google Scholar 

  7. Stasi R, Stipa E, Masi M, Cecconi M, Scimo MT, Oliva F, et al. Long-term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med. 1995a;98(5):436–42.

    Article  CAS  PubMed  Google Scholar 

  8. Neunert C, Noroozi N, Norman G, Buchanan GR, Goy J, Nazi I, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost. 2015;13(3):457–64.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Frederiksen H, Maegbaek ML, Norgaard M. Twenty-year mortality of adult patients with primary immune thrombocytopenia: a Danish population-based cohort study. Br J Haematol. 2014;166(2):260–7.

    Article  PubMed  Google Scholar 

  10. Norgaard M, Jensen AO, Engebjerg MC, Farkas DK, Thomsen RW, Cha S, et al. Long-term clinical outcomes of patients with primary chronic immune thrombocytopenia: a Danish population-based cohort study. Blood. 2011;117(13):3514–20.

    Article  PubMed  Google Scholar 

  11. Schoonen WM, Kucera G, Coalson J, Li L, Rutstein M, Mowat F, et al. Epidemiology of immune thrombocytopenic purpura in the General Practice Research Database. Br J Haematol. 2009;145(2):235–44.

    Article  PubMed  Google Scholar 

  12. Rodeghiero F, Michel M, Gernsheimer T, Ruggeri M, Blanchette V, Bussel JB, et al. Standardization of bleeding assessment in immune thrombocytopenia: report from the International Working Group. Blood. 2013a;121(14):2596–606.

    Article  CAS  PubMed  Google Scholar 

  13. Crosby WH. Editorial: wet purpura, dry purpura. JAMA. 1975;232(7):744–5.

    Article  CAS  PubMed  Google Scholar 

  14. Neunert CE. Individualized treatment for immune thrombocytopenia: predicting bleeding risk. Semin Hematol. 2013;50(Suppl 1):S55–7.

    Article  PubMed  Google Scholar 

  15. 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(11):1630–8.

    Article  CAS  PubMed  Google Scholar 

  16. Rodeghiero F. Is ITP a thrombophilic disorder? Am J Hematol. 2016;91(1):39–45.

    Article  PubMed  Google Scholar 

  17. Enger C, Bennett D, Forssen U, Fogarty PF, McAfee AT. Comorbidities in patients with persistent or chronic immune thrombocytopenia. Int J Hematol. 2010;92(2):289–95.

    Article  PubMed  Google Scholar 

  18. Nørgaard M, Cetin K, Maegbaek ML, Kristensen NR, Ghanima W, Bahmanyar S, et al. Risk of arterial thrombotic and venous thromboembolic events in patients with primary chronic immune thrombocytopenia: a Scandinavian population-based cohort study. Br J Haematol. 2015;174(4):639–42.

    Article  PubMed  Google Scholar 

  19. Nørgaard M, Severinsen MT, Lund Maegbaek M, Jensen A, Cha S, Sørensen HT. Risk of arterial thrombosis in patients with primary chronic immune thrombocytopenia: a Danish population-based cohort study. Br J Haematol. 2012;159(1):109–11.

    Article  PubMed  Google Scholar 

  20. Sarpatwari A, Bennett D, Logie JW, Shukla A, Beach KJ, Newland AC, et al. Thromboembolic events among adult patients with primary immune thrombocytopenia in the United Kingdom General Practice Research Database. Haematologica. 2010;95(7):1167–75.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Zoller B, Li X, Sundquist J, Sundquist K. Risk of pulmonary embolism in patients with autoimmune disorders: a nationwide follow-up study from Sweden. Lancet. 2012;379(9812):244–9.

    Article  PubMed  Google Scholar 

  22. Langeberg WJ, Schoonen WM, Eisen M, Gamelin L, Stryker S. Thromboembolism in patients with immune thrombocytopenia (ITP): a meta-analysis of observational studies. Int J Hematol. 2016;103(6):655–64.

    Article  CAS  PubMed  Google Scholar 

  23. Sewify EM, Sayed D, Abdel Aal RF, Ahmad HM, Abdou MA. Increased circulating red cell microparticles (RMP) and platelet microparticles (PMP) in immune thrombocytopenic purpura. Thromb Res. 2013;131(2):e59–63.

    Article  CAS  PubMed  Google Scholar 

  24. Kosugi S, Tomiyama Y, Honda S, Kashiwagi H, Shiraga M, Tadokoro S, et al. Anti-alphavbeta3 antibodies in chronic immune thrombocytopenic purpura. Thromb Haemost. 2001;85(1):36–41.

    CAS  PubMed  Google Scholar 

  25. Kim KJ, Baek IW, Yoon CH, Kim WU, Cho CS. Thrombotic risk in patients with immune thrombocytopenia and its association with antiphospholipid antibodies. Br J Haematol. 2013;161(5):706–14.

    Article  CAS  PubMed  Google Scholar 

  26. Fardet L, Feve B. Systemic glucocorticoid therapy: a review of its metabolic and cardiovascular adverse events. Drugs. 2014;74(15):1731–45.

    Article  CAS  PubMed  Google Scholar 

  27. Marcen R. Immunosuppressive drugs in kidney transplantation: impact on patient survival, and incidence of cardiovascular disease, malignancy and infection. Drugs. 2009;69(16):2227–43.

    Article  CAS  PubMed  Google Scholar 

  28. Sorensen HT, Mellemkjaer L, Nielsen GL, Baron JA, Olsen JH, Karagas MR. Skin cancers and non-hodgkin lymphoma among users of systemic glucocorticoids: a population-based cohort study. J Natl Cancer Inst. 2004;96(9):709–11.

    Article  PubMed  Google Scholar 

  29. Boyle S, White RH, Brunson A, Wun T. Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia. Blood. 2013;121(23):4782–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Thomsen RW, Schoonen WM, Farkas DK, Riis A, Jacobsen J, Fryzek JP, et al. Risk for hospital contact with infection in patients with splenectomy: a population-based cohort study. Ann Intern Med. 2009;151(8):546–55.

    Article  PubMed  Google Scholar 

  31. McMillan R, Bussel JB, George JN, Lalla D, Nichol JL. Self-reported health-related quality of life in adults with chronic immune thrombocytopenic purpura. Am J Hematol. 2008;83(2):150–4.

    Article  PubMed  Google Scholar 

  32. Snyder CF, Mathias SD, Cella D, Isitt JJ, Wu AW, Young J. Health-related quality of life of immune thrombocytopenic purpura patients: results from a web-based survey. Curr Med Res Opin. 2008;24(10):2767–76.

    Article  PubMed  Google Scholar 

  33. Hill QA, Newland AC. Fatigue in immune thrombocytopenia. Br J Haematol. 2015;170(2):141–9.

    Article  PubMed  Google Scholar 

  34. Cheng G, Saleh MN, Marcher C, Vasey S, Mayer B, Aivado M, et al. Eltrombopag for management of chronic immune thrombocytopenia (RAISE): a 6-month, randomised, phase 3 study. Lancet. 2011;377(9763):393–402.

    Article  CAS  PubMed  Google Scholar 

  35. Kuter DJ, Rummel M, Boccia R, Macik BG, Pabinger I, Selleslag D, et al. Romiplostim or standard of care in patients with immune thrombocytopenia. N Engl J Med. 2010;363(20):1889–99.

    Article  CAS  PubMed  Google Scholar 

  36. Kuwana M. Helicobacter pylori-associated immune thrombocytopenia: clinical features and pathogenic mechanisms. World J Gastroenterol. 2014;20(3):714–23.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Fujimura K, Miyakawa Y, Kurata Y, Kuwana M, Tomiyama Y, Murata M. Reference guide for management of adult idiopathic thrombocytopenic purpura (ITP) 2012 version. Rinsho Ketsueki. 2012;53(4):433–42.

    PubMed  Google Scholar 

  38. Stasi R, Sarpatwari A, Segal JB, Osborn J, Evangelista ML, Cooper N, et al. Effects of eradication of Helicobacter pylori infection in patients with immune thrombocytopenic purpura: a systematic review. Blood. 2009;113(6):1231–40.

    Article  CAS  PubMed  Google Scholar 

  39. 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(4):574–96.

    Article  Google Scholar 

  40. George JN, Woolf SH, Raskob GE, Wasser JS, Aledort LM, Ballem PJ, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood. 1996;88(1):3–40.

    CAS  PubMed  Google Scholar 

  41. Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Crowther MA, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117(16):4190–207.

    Article  CAS  PubMed  Google Scholar 

  42. Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–86.

    Article  CAS  PubMed  Google Scholar 

  43. Cheng Y, Wong RS, Soo YO, Chui CH, Lau FY, Chan NP, et al. Initial treatment of immune thrombocytopenic purpura with high-dose dexamethasone. N Engl J Med. 2003;349(9):831–6.

    Article  CAS  PubMed  Google Scholar 

  44. Mazzucconi MG, Fazi P, Bernasconi S, De Rossi G, Leone G, Gugliotta L, et al. Therapy with high-dose dexamethasone (HD-DXM) in previously untreated patients affected by idiopathic thrombocytopenic purpura: a GIMEMA experience. Blood. 2007;109(4):1401–7.

    Article  CAS  PubMed  Google Scholar 

  45. Wei, Y., Ji, X. B., Wang, Y. W., Wang, J. X., Yang, E. Q., Wang, Z. C., et al. (2016). High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial. Blood, 127(3), 296-302; quiz 370.

    Google Scholar 

  46. Kojouri K, Vesely SK, Terrell DR, George JN. 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.

    Article  CAS  PubMed  Google Scholar 

  47. Gonzalez-Porras JR, Escalante F, Pardal E, Sierra M, Garcia-Frade LJ, Redondo S, et al. Safety and efficacy of splenectomy in over 65-yrs-old patients with immune thrombocytopenia. Eur J Haematol. 2013;91(3):236–41.

    Article  PubMed  Google Scholar 

  48. Tomiyama Y. Thrombopoietin receptor agonists. In: Salama A, editor. Current & emerging treatments for immune thrombocytopenia. London: Future Medicine, Ltd.; 2013. p. 89–100.

    Google Scholar 

  49. Bussel JB, Provan D, Shamsi T, Cheng G, Psaila B, Kovaleva L, et al. Effect of eltrombopag on platelet counts and bleeding during treatment of chronic idiopathic thrombocytopenic purpura: a randomised, double-blind, placebo-controlled trial. Lancet. 2009;373(9664):641–8.

    Article  CAS  PubMed  Google Scholar 

  50. Kuter DJ, Bussel JB, Lyons RM, Pullarkat V, Gernsheimer TB, Senecal FM, et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. Lancet. 2008;371(9610):395–403.

    Article  CAS  PubMed  Google Scholar 

  51. Rodeghiero F, Stasi R, Giagounidis A, Viallard JF, Godeau B, Pabinger I, et al. Long-term safety and tolerability of romiplostim in patients with primary immune thrombocytopenia: a pooled analysis of 13 clinical trials. Eur J Haematol. 2013b;91(5):423–36.

    Article  CAS  PubMed  Google Scholar 

  52. Saleh MN, Bussel JB, Cheng G, Meyer O, Bailey CK, Arning M, et al. Safety and efficacy of eltrombopag for treatment of chronic immune thrombocytopenia: results of the long-term, open-label EXTEND study. Blood. 2013;121(3):537–45.

    Article  CAS  PubMed  Google Scholar 

  53. Sailler L. Rituximab off label use for difficult-to-treat auto-immune diseases: reappraisal of benefits and risks. Clin Rev Allergy Immunol. 2008;34(1):103–10.

    Article  CAS  PubMed  Google Scholar 

  54. Patel VL, Mahevas M, Lee SY, Stasi R, Cunningham-Rundles S, Godeau B, et al. Outcomes 5 years after response to rituximab therapy in children and adults with immune thrombocytopenia. Blood. 2012;119(25):5989–95.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Cuker A, Neunert CE. How I treat refractory immune thrombocytopenia. Blood. 2016;128(12):1547–54.

    Article  PubMed  Google Scholar 

  56. Alpdogan O, Budak-Alpdogan T, Ratip S, Firatli-Tuglular T, Tanriverdi S, Karti S, et al. Efficacy of high-dose methylprednisolone as a first-line therapy in adult patients with idiopathic thrombocytopenic purpura. Br J Haematol. 1998;103(4):1061–3.

    Article  CAS  PubMed  Google Scholar 

  57. Abrahm J, Ellman L. Platelet transfusion in immune thrombocytopenic purpura. JAMA. 1976;236(16):1847.

    Article  CAS  PubMed  Google Scholar 

  58. Spahr JE, Rodgers GM. Treatment of immune-mediated thrombocytopenia purpura with concurrent intravenous immunoglobulin and platelet transfusion: a retrospective review of 40 patients. Am J Hematol. 2008;83(2):122–5.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgment

This work was supported by Grant-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology and the Ministry of Health, Labour and Welfare in Japan.

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Correspondence to Hirokazu Kashiwagi M.D. .

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Kashiwagi, H., Tomiyama, Y. (2017). ITP in Adults. In: Ishida, Y., Tomiyama, Y. (eds) Autoimmune Thrombocytopenia . Springer, Singapore. https://doi.org/10.1007/978-981-10-4142-6_7

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  • DOI: https://doi.org/10.1007/978-981-10-4142-6_7

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