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Background of Immune Thrombocytopenia

  • Kristina M. Haley
Chapter

Abstract

Immune thrombocytopenia (ITP) is a heterogeneous disease characterized by immune-mediated thrombocytopenia and associated with variable mucocutaneous bleeding. Historically, symptoms associated with ITP have been described for years. However, until the platelet was defined in the 1800s, the link to thrombocytopenia was unknown. From that point forward, the etiology of thrombocytopenia was debated and attributed at various times in history to increased platelet destruction and/or to decreased platelet production. The diagnosis is one of exclusion, and inherited and acquired causes of thrombocytopenia should be included in the differential diagnosis. In this chapter, the history of ITP, the terminology used in ITP, and the clinical presentation with differential diagnosis will be discussed.

Keywords

Immune thrombocytopenia Complete response Congenital amegakaryocytic thrombocytopenia Platelet size Inherited vs. acquired 

References

  1. 1.
    Cuker A, Cines DB. Immune thrombocytopenia. Hematol Am Soc Hematol Educ Prog. 2010;2010:377–84.Google Scholar
  2. 2.
    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;115:168–86.CrossRefPubMedGoogle Scholar
  3. 3.
    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;113:2386–93.CrossRefPubMedGoogle Scholar
  4. 4.
    Terrell DR, Beebe LA, Vesely SK, Neas BR, Segal JB, George JN. The incidence of immune thrombocytopenic purpura in children and adults: a critical review of published reports. Am J Hematol. 2010;85:174–80.CrossRefPubMedGoogle Scholar
  5. 5.
    Neunert CE. Current management of immune thrombocytopenia. Hematol Am Soc Hematol Educ Prog. 2013;2013:276–82.Google Scholar
  6. 6.
    Cooper N. State of the art—how I manage immune thrombocytopenia. Br J Haematol. 2017;177:39–54.CrossRefPubMedGoogle Scholar
  7. 7.
    Kistangari G, Mccrae KR. Immune thrombocytopenia. Hematol Oncol Clin North Am. 2013;27:495–520.CrossRefPubMedGoogle Scholar
  8. 8.
    Cines DB, Bussel JB, Liebman HA, Luning Prak ET. The ITP syndrome: pathogenic and clinical diversity. Blood. 2009;113:6511–21.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Stasi R, Newland AC. ITP: a historical perspective. Br J Haematol. 2011;153:437–50.CrossRefPubMedGoogle Scholar
  10. 10.
    Blanchette M, Freedman J. The history of idiopathic thrombocytopenic purpura (ITP). Transfus Sci. 1998;19:231–6.CrossRefPubMedGoogle Scholar
  11. 11.
    Brewer DB. Max Schultze (1865), G. Bizzozero (1882) and the discovery of the platelet. Br J Haematol. 2006;133:251–8.CrossRefPubMedGoogle Scholar
  12. 12.
    Anoop P. Immune thrombocytopenic purpura: historical perspective, current status, recent advances and future directions. Indian Pediatr. 2012;49:811–8.CrossRefPubMedGoogle Scholar
  13. 13.
    Liebman HA. Immune thrombocytopenia (ITP): an historical perspective. Hematol Am Soc Hematol Educ Prog. 2008;2008:205.Google Scholar
  14. 14.
    Dameshek W, Miller EB. The megakaryocytes in idiopathic thrombocytopenic purpura, a form of hypersplenism. Blood. 1946;1:27–50.PubMedPubMedCentralGoogle Scholar
  15. 15.
    Stoll D, Cines DB, Aster RH, Murphy S. Platelet kinetics in patients with idiopathic thrombocytopenic purpura and moderate thrombocytopenia. Blood. 1985;65:584–8.PubMedGoogle Scholar
  16. 16.
    Mcmillan R, Wang L, Tomer A, Nichol J, Pistillo J. Suppression of in vitro megakaryocyte production by antiplatelet autoantibodies from adult patients with chronic ITP. Blood. 2004;103:1364–9.CrossRefPubMedGoogle Scholar
  17. 17.
    Chang M, Nakagawa PA, Williams SA, Schwartz MR, Imfeld KL, Buzby JS, et al. Immune thrombocytopenic purpura (ITP) plasma and purified ITP monoclonal autoantibodies inhibit megakaryocytopoiesis in vitro. Blood. 2003;102:887–95.CrossRefPubMedGoogle Scholar
  18. 18.
    Houwerzijl EJ, Blom NR, Van Der Want JJ, Esselink MT, Koornstra JJ, Smit JW, et al. Ultrastructural study shows morphologic features of apoptosis and para-apoptosis in megakaryocytes from patients with idiopathic thrombocytopenic purpura. Blood. 2004;103:500–6.CrossRefPubMedGoogle Scholar
  19. 19.
    Olsson B, et al. T-cell-mediated cytotoxicity toward platelets in chronic idiopathic thrombocytopenic purpura. Nature Medicine. 2003;9(9):1123–24.CrossRefPubMedGoogle Scholar
  20. 20.
    Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA, American Society of Hematology. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117:4190–207.CrossRefPubMedGoogle Scholar
  21. 21.
    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;13:457–64.CrossRefPubMedGoogle Scholar
  22. 22.
    Imbach P, et al. Childhood ITP: 12 months follow-up data from the prospective registry of the Intercontinental Childhood ITP Study Group (ICIS). 2006;46:351–56.Google Scholar
  23. 23.
    Grace RF, Long M, Kalish LA, Neufeld EJ. Applicability of 2009 international consensus terminology and criteria for immune thrombocytopenia to a clinical pediatric population. Pediatr Blood Cancer. 2012;58:216–20.CrossRefGoogle Scholar
  24. 24.
    Cines DB, Bussel JB. How I treat idiopathic thrombocytopenic purpura (ITP). Blood. 2005;106:2244–51.CrossRefPubMedGoogle Scholar
  25. 25.
    Nugent DJ. Immune thrombocytopenic purpura of childhood. Hematol Am Soc Hematol Educ Prog. 2006;2006:97–103.CrossRefGoogle Scholar
  26. 26.
    Lo E, Deane S. Diagnosis and classification of immune-mediated thrombocytopenia. Autoimmun Rev. 2014;13:577–83.CrossRefPubMedGoogle Scholar
  27. 27.
    Cines DB, Liebman H, Stasi R. Pathobiology of secondary immune thrombocytopenia. Semin Hematol. 2009;46:S2–14.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Ayesh MH, Alawneh K, Khassawneh B, Khader Y, Kasasbeh A. Adult primary and secondary immune thrombocytopenic purpura: a comparative analysis of characteristics and clinical course. Clin Appl Thromb Hemost. 2013;19:327–30.CrossRefPubMedGoogle Scholar
  29. 29.
    Balduini CL, Cattaneo M, Fabris F, Gresele P, Iolascon A, Pulcinelli FM, et al. Inherited thrombocytopenias: a proposed diagnostic algorithm from the Italian Gruppo di Studio delle Piastrine. Haematologica. 2003;88:582–92.Google Scholar
  30. 30.
    Lambert MP. What to do when you suspect an inherited platelet disorder. Hematol Am Soc Hematol Educ Prog. 2011;2011:377–83.CrossRefPubMedGoogle Scholar
  31. 31.
    Stasi R. How to approach thrombocytopenia. Hematol Am Soc Hematol Educ Prog. 2012;2012:191–7.Google Scholar
  32. 32.
    Gauer RL, Braun MM. Thrombocytopenia. Am Fam Physician. 2012;85:612–22.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatric Hematology/Oncology, Department of PediatricsOregon Health and Science UniversityPortlandUSA

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