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Annals of Hematology

, Volume 89, Supplement 1, pp 11–17 | Cite as

Diagnosis and management of chronic ITP: comments from an ICIS expert group

  • John David Grainger
  • Paula H. B. Bolton-Maggs
  • Bertrand Godeau
  • Jim Bussel
  • Hugo Donato
  • Mohsen Elalfy
  • Ina Hainmann
  • Axel Matzdorff
  • Hannes Müller-Beissenhirtz
  • Alicia Rovó
  • Andre Tichelli
Review Article

Abstract

Immune thrombocytopenia (ITP) is a common disorder in children and adults. In a patient with newly diagnosed ITP, the treatment strategy is relatively well defined. Second-line treatments are more controversial, and the management of chronic ITP is even more so. During the 3rd ICIS Expert Meeting on Consensus and Development of Strategies in ITP, held in Basel on September 3–5, 2009, a group of experts were tasked with reaching a consensus on some frequently asked questions relating to diagnosis and management of children and adults with chronic ITP. The content of this article is designed to provide a practical support to trained haematologists in their care of patients with chronic ITP.

Keywords

Immune thrombocytopenia ITP 

Notes

Acknowledgements

We would like to thank all participants in the chronic ITP expert group who participated in the discussion at ICIS, namely: Bessho, Fumio—Moroyama, Japan; Diesch, Tamara—Basel, Switzerland; Erduran, Erol—Trabzon, Turkey; Fouda, Ashraf—Al-Mansoura, Egypt; Hainmann, Ina—Freiburg, Germany; Kaplinsky, Chaim—Tel Hashomer, Israel; Konja, Josip—Zagreb, Croatia; Kreuzbauer, Georg—Zug, Switzerland; Kroiss, Sabine—Zürich, Switzerland; Strauss, Gabriele—Berlin, Germany; Tamminga, Rienk—Groningen, Germany; and Veronese, Luisa—London, UK.

References

  1. 1.
    Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, Bussel JB, Cines DB, Chong BH, Cooper N, Godeau B, Lechner K, Mazzucconi MG, McMillan R, Sanz MA, Imbach P, Blanchette V, Kühne T, Ruggeri M, George JN (2009) Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 113(11):2386–2393CrossRefPubMedGoogle Scholar
  2. 2.
    George JN, Woolf SH, Raskob GE et al (1996) Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods of American Society of Hematology. Blood 88:3–40PubMedGoogle Scholar
  3. 3.
    British Committee for Standards in Haematology General Haematology Task Force (2003) Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol 120:574–596CrossRefGoogle Scholar
  4. 4.
    Kuter DJ, Mufti GJ, Bain BJ, Hasserjian RP, Davis W, Rutstein M (2009) Evaluation of bone marrow reticulin formation in chronic immune thrombocytopenia (ITP) patients treated with romiplostim. Blood 114(18):3748–3756CrossRefPubMedGoogle Scholar
  5. 5.
    Rice L (2009) Treatment of immune thrombocytopenic purpura: focus on eltrombopag. Biologics 3:151–157PubMedGoogle Scholar
  6. 6.
    Jamali F, Lemery S, Ayalew K, Robottom S, Robie-Suh K, Rieves D, Pazdur R (2009) Romiplostim for the treatment of chronic immune (idiopathic) thrombocytopenic purpura. Oncology (Williston Park) 23(8):704–709Google Scholar
  7. 7.
    Brighton TA, Evans S, Castaldi PA, Chesterman CN, Chong BH (1996) Prospective evaluation of the clinical usefulness of an antigen-specific assay (MAIPA) in idiopathic thrombocytopenic purpura and other immune thrombocytopenias. Blood 88:194–201PubMedGoogle Scholar
  8. 8.
    Fabris F, Scandellari R, Ruzzon E, Randi ML, Luzzatto G, Girolami A (2004) Platelet-associated autoantibodies as detected by a solid-phase modified antigen capture ELISA test (MACE) are a useful prognostic factor in idiopathic thrombocytopenic purpura. Blood 103:4562–4564CrossRefPubMedGoogle Scholar
  9. 9.
    Sikorska A, Konopka L, Maslanka K (2008) The effect of platelet autoantobodies on the course of the disease and clinical response of patients with idiopathic thrombocytopenic purpura. Int Jnl Lab Hem 30:58–64CrossRefGoogle Scholar
  10. 10.
    Cines DB, Bussel JB, Liebman HA, Luning Prak ET (2009) The ITP syndrome: pathogenic and clinical diversity. Blood 113:6511–6521CrossRefPubMedGoogle Scholar
  11. 11.
    Liebman HA (2008) Viral-associated immune thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program 2008(1):212–218Google Scholar
  12. 12.
    Scaradavou A, Cunningham-Rundles S, Ho JL, Folman C, Doo H, Bussel JB (2007) Superior effect of intravenous anti-D compared with IV gammaglobulin in the treatment of HIV-thrombocytopenia: results of a small, randomized prospective comparison. Am J Hematol 82(5):335–341CrossRefPubMedGoogle Scholar
  13. 13.
    Carson KR, Evens AM, Richey EA, Habermann TM, Focosi D, Seymour JF, Laubach J, Bawn SD, Gordon LI, Winter JN, Furman RR, Vose JM, Zelenetz AD, Mamtani R, Raisch DW, Dorshimer GW, Rosen ST, Muro K, Gottardi-Littell NR, Talley RL, Sartor O, Green D, Major EO, Bennett CL (2009) Progressive multifocal leukoencephalopathy after rituximab therapy in HIV-negative patients: a report of 57 cases from the Research on Adverse Drug Events and Reports project. Blood 113(20):4834–4840CrossRefPubMedGoogle Scholar
  14. 14.
    Norton A, Roberts I (2005) Management of Evans syndrome. Br J Haematol 132:125–137CrossRefGoogle Scholar
  15. 15.
    Stasi R, Stipa E, Masi M et al (1994) Prevalence and clinical significance of elevated antiphospholipid antibodies in patients with idiopathic thrombocytopenic purpura. Blood 84:4203–4208PubMedGoogle Scholar
  16. 16.
    Psaila B, Petrovic A, Page LK, Menell J, Schonholz M, Bussel JB (2009) Intracranial hemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases. Blood 114(23):4777–4783CrossRefPubMedGoogle Scholar
  17. 17.
    Butros LJ (2003) Bussel JB Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis. J Pediatr Hematol Oncol 25(8):660–664CrossRefPubMedGoogle Scholar
  18. 18.
    Page KL, Psaila B, Provan D et al (2007) The immune thrombocytopenic purpura (ITP) bleeding score: assessment of bleeding in patients with ITP. Br J Haematol 138:245–248CrossRefPubMedGoogle Scholar
  19. 19.
    Khellaf M, Michel M, Schaeffer A, Bierling P, Godeau B (2005) Assessment of a therapeutic strategy for adults with severe autoimmune thrombocytopenic purpura based on a bleeding score rather than platelet count. Haematologica 90(6):829–832PubMedGoogle Scholar
  20. 20.
    Panzer S, Rieger M, Vormittag R, Eichelberger B, Dunkler D, Pabinger I (2007) Platelet function to estimate the bleeding risk in autoimmune thrombocytopenia. Eur J Clin Invest 37(10):814–819CrossRefPubMedGoogle Scholar
  21. 21.
    Michiels JJ, Berneman Z, Gadisseur A, van der Planken M, Schroyens W, van Vliet HH (2009) Laboratory diagnosis and molecular basis of mild von Willebrand disease type 1. Acta Haematol 121(2–3):85–97CrossRefPubMedGoogle Scholar
  22. 22.
    Sucker C, Michiels JJ, Zotz RB (2009) Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies. Acta Haematol 121(2–3):177–182CrossRefPubMedGoogle Scholar
  23. 23.
    Kojouri K, Vesely SK, Terrell DR, George JN (2004) 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 104(9):2623–2634CrossRefPubMedGoogle Scholar
  24. 24.
    Schilling RF, Gangnon RE, Traver MI (2008) Delayed adverse vascular events after splenectomy in hereditary spherocytosis. J Thromb Haemost 6(8):1289CrossRefPubMedGoogle Scholar
  25. 25.
    Bennett CM, Rogers ZR, Kinnamon DD, Bussel JB, Mahoney DH, Abshire TC, Sawaf H, Moore TB, Loh ML, Glader BE, McCarthy MC, Mueller BU, Olson TA, Lorenzana AN, Mentzer WC, Buchanan GR, Feldman HA, Neufeld EJ (2006) Prospective phase 1/2 study of rituximab in childhood and adolescent chronic immune thrombocytopenic purpura. Blood 107(7):2639–2642CrossRefPubMedGoogle Scholar
  26. 26.
    Carson KR, Evens AM, Richey EA, Habermann TM, Focosi D, Seymour JF et al (2009) Progressive multifocal leukoencephalopathy following rituximab therapy in HIV negative patients: a report of 57 cases from the Research on Adverse Drug Event and Reports (RADAR) project. Blood 113(20):4834–4840CrossRefPubMedGoogle Scholar
  27. 27.
    Bussel JB, Provan D, Shamsi T, Cheng G, Psaila B, Kovaleva L, Salama A, Jenkins JM, Roychowdhury D, Mayer B, Stone N, Arning M (2009) Effect of eltrombopag on platelet counts and bleeding during treatment of chronic idiopathic thrombocytopenic purpura: a randomised, double-blind, placebo-controlled trial. Lancet 373(9664):641–648CrossRefPubMedGoogle Scholar
  28. 28.
    Kuter DJ, Bussel JB, Lyons RM, Pullarkat V, Gernsheimer TB, Senecal FM, Aledort LM, George JN, Kessler CM, Sanz MA, Liebman HA, Slovick FT, de Wolf JT, Bourgeois E, Guthrie TH Jr, Newland A, Wasser JS, Hamburg SI, Grande C, Lefrère F, Lichtin AE, Tarantino MD, Terebelo HR, Viallard JF, Cuevas FJ, Go RS, Henry DH, Redner RL, Rice L, Schipperus MR, Guo DM, Nichol JL (2008) Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. Lancet 371(9610):395–403CrossRefPubMedGoogle Scholar
  29. 29.
    Kuter DJ, Mufti GJ, Bain BJ, Hasserjian RP, Davis W, Rutstein M (2009) Evaluation of bone marrow reticulin formation in chronic immune thrombocytopenia patients treated with romiplostim. Blood 114(18):3748–3756CrossRefPubMedGoogle Scholar
  30. 30.
    Bruhn HD, Schambeck CM, Hach-Wunderle V (2007) Hämostaseologie für die Praxis. Schattauer GmbH, StuttgartGoogle Scholar
  31. 31.
    Kobrinsky NL, Tulloch H (1988) Treatment of refractory thrombocytopenic bleeding with 1-desamino-8-D-arginine vasopressin (desmopressin). J Pediatr 112(6):993–996CrossRefPubMedGoogle Scholar
  32. 32.
    Valeri CR, Cassidy G, Pivacek LE, Ragno G, Lieberthal W, Crowley JP, Khuri SF, Loscalzo J (2001) Anemia-induced increase in the bleeding time: implications for treatment of nonsurgical blood loss. Transfusion 41(8):977–983CrossRefPubMedGoogle Scholar
  33. 33.
    Beatty MN, Blumenthal PD (2009) The levonorgestrel-releasing intrauterine system: safety, efficacy, and patient acceptability. Ther Clin Risk Manag 5(3):561–574PubMedGoogle Scholar
  34. 34.
    Fruchter O, Blich M, Jacob G (2002) Fatal acute myocardial infarction during severe thrombocytopenia in a patient with idiopathic thrombocytopenic purpura. Am J Med Sci 323:279–280CrossRefPubMedGoogle Scholar
  35. 35.
    Gracia MC, Cebollero IC, Lezcano JS et al (2008) Invasive treatment performed for acute myocardial infarction in a patient with immune thrombocytopenic purpura. Int J Cardiol 127:e183–e185CrossRefPubMedGoogle Scholar
  36. 36.
    Theeler BJ, Ney JP (2008) A patient with idiopathic thrombocytopenic purpura presenting with an acute ischemic stroke. J Stroke Cerebrovasc Dis 17:244–245CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • John David Grainger
    • 1
  • Paula H. B. Bolton-Maggs
    • 1
  • Bertrand Godeau
    • 5
  • Jim Bussel
    • 2
  • Hugo Donato
    • 3
  • Mohsen Elalfy
    • 4
  • Ina Hainmann
    • 6
  • Axel Matzdorff
    • 7
  • Hannes Müller-Beissenhirtz
    • 8
  • Alicia Rovó
    • 9
  • Andre Tichelli
    • 9
  1. 1.ManchesterUK
  2. 2.New YorkUSA
  3. 3.San JustoArgentina
  4. 4.CairoEgypt
  5. 5.ParisFrance
  6. 6.FreiburgGermany
  7. 7.SaarbrückenGermany
  8. 8.EssenGermany
  9. 9.BaselSwitzerland

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