Skip to main content
SpringerLink
Log in

Diagnosis in General

  • Chapter
  • First Online:
Autoimmune Thrombocytopenia

Abstract

Diagnosis of ITP is carried out by mainly exclusion of thrombocytopenia caused by other etiology and therapeutic responses. Since thrombocytopenia may be associated with a variety of clinical conditions, careful exclusion is necessary. Blood smear examination is especially important to rule out pseudothrombocytopenia and some congenital thrombocytopenia. Bone marrow examination may not be essential to ITP diagnosis, but it should be performed in cases with abnormalities in CBC or blood smear other than thrombocytopenia, atypical clinical course, or refractory to standard treatments. Assessment of reticulated platelets and plasma thrombopoietin concentration may be helpful to distinguish ITP from hypoplastic thrombocytopenia.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 179.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Kashiwagi H, Tomiyama Y. Pathophysiology and management of primary immune thrombocytopenia. Int J Hematol. 2013;98(1):24–33.

    Article  CAS  PubMed  Google Scholar 

  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(2):168–86.

    Article  CAS  PubMed  Google Scholar 

  3. Berkman N, Michaeli Y, Or R, Eldor A. EDTA-dependent pseudothrombocytopenia: a clinical study of 18 patients and a review of the literature. Am J Hematol. 1991;36(3):195–201.

    Article  CAS  PubMed  Google Scholar 

  4. Kunishima S, Saito H. Advances in the understanding of MYH9 disorders. Curr Opin Hematol. 2010;17(5):405–10.

    Article  CAS  PubMed  Google Scholar 

  5. Bolton-Maggs PH, Chalmers EA, Collins PW, Harrison P, Kitchen S, Liesner RJ, et al. A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO. Br J Haematol. 2006;135(5):603–33.

    Article  CAS  PubMed  Google Scholar 

  6. 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 

  7. Stasi R, Stipa E, Masi M, Oliva F, Sciarra A, Perrotti A, et al. Prevalence and clinical significance of elevated antiphospholipid antibodies in patients with idiopathic thrombocytopenic purpura. Blood. 1994;84(12):4203–8.

    CAS  PubMed  Google Scholar 

  8. Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia. 2008;14(2):171–232.

    Article  CAS  PubMed  Google Scholar 

  9. Liebman HA, Stasi R. Secondary immune thrombocytopenic purpura. Curr Opin Hematol. 2007;14(5):557–73.

    Article  PubMed  Google Scholar 

  10. Stasi R, Willis F, Shannon MS, Gordon-Smith EC. Infectious causes of chronic immune thrombocytopenia. Hematol Oncol Clin North Am. 2009;23(6):1275–97.

    Article  PubMed  Google Scholar 

  11. 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 

  12. Karpatkin S, Nardi MA, Hymes KB. Sequestration of anti-platelet GPIIIa antibody in rheumatoid factor immune complexes of human immunodeficiency virus 1 thrombocytopenic patients. Proc Natl Acad Sci U S A. 1995;92(6):2263–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Lacey JV, Penner JA. Management of idiopathic thrombocytopenic purpura in the adult. Semin Thromb Hemost. 1977;3(3):160–74.

    CAS  PubMed  Google Scholar 

  14. Jubelirer SJ, Harpold R. The role of the bone marrow examination in the diagnosis of immune thrombocytopenic purpura: case series and literature review. Clin Appl Thromb Hemost. 2002;8(1):73–6.

    Article  PubMed  Google Scholar 

  15. Mahabir VK, Ross C, Popovic S, Sur ML, Bourgeois J, Lim W, et al. A blinded study of bone marrow examinations in patients with primary immune thrombocytopenia. Eur J Haematol. 2013;90(2):121–6.

    Article  PubMed  Google Scholar 

  16. 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 

  17. Kantarjian H, Fenaux P, Sekeres MA, Becker PS, Boruchov A, Bowen D, et al. Safety and efficacy of romiplostim in patients with lower-risk myelodysplastic syndrome and thrombocytopenia. J Clin Oncol. 2010;28(3):437–44.

    Article  CAS  PubMed  Google Scholar 

  18. Oshima Y, Yuji K, Tanimoto T, Hinomura Y, Tojo A. Association between acute myelogenous leukemia and thrombopoietin receptor agonists in patients with immune thrombocytopenia. Intern Med. 2013;52(19):2193–201.

    Article  PubMed  Google Scholar 

  19. Prica A, Sholzberg M, Buckstein R. Safety and efficacy of thrombopoietin-receptor agonists in myelodysplastic syndromes: a systematic review and meta-analysis of randomized controlled trials. Br J Haematol. 2014;167(5):626–38.

    Article  CAS  PubMed  Google Scholar 

  20. Najean Y, Lecompte T. Chronic pure thrombocytopenia in elderly patients. An aspect of the myelodysplastic syndrome. Cancer. 1989;64(12):2506–10.

    Article  CAS  PubMed  Google Scholar 

  21. Gupta R, Soupir CP, Johari V, Hasserjian RP. Myelodysplastic syndrome with isolated deletion of chromosome 20q: an indolent disease with minimal morphological dysplasia and frequent thrombocytopenic presentation. Br J Haematol. 2007;139(2):265–8.

    Article  PubMed  Google Scholar 

  22. Mittal S, Blaylock MG, Culligan DJ, Barker RN, Vickers MA. A high rate of CLL phenotype lymphocytes in autoimmune hemolytic anemia and immune thrombocytopenic purpura. Haematologica. 2008;93(1):151–2.

    Article  PubMed  Google Scholar 

  23. Heitink-Polle KM, Nijsten J, Boonacker CW, de Haas M, Bruin MC. Clinical and laboratory predictors of chronic immune thrombocytopenia in children: a systematic review and meta-analysis. Blood. 2014;124(22):3295–307.

    Article  CAS  PubMed  Google Scholar 

  24. Artim-Esen B, Diz-Kucukkaya R, Inanc M. The significance and management of thrombocytopenia in antiphospholipid syndrome. Curr Rheumatol Rep. 2015;17(3):14.

    Article  PubMed  Google Scholar 

  25. Meyer O. Symptoms and diagnosis. In: Salama A, editor. Current & emerging treatments for immune thrombocytopenia. London: Future Medicine; 2013. p. 19–29.

    Google Scholar 

  26. Kelton JG, Murphy WG, Lucarelli A, Garvey-Williams J, Santos A, Meyer R, et al. A prospective comparison of four techniques for measuring platelet-associated IgG. Br J Haematol. 1989;71(1):97–105.

    Article  CAS  PubMed  Google Scholar 

  27. Mueller-Eckhardt C, Kayser W, Mersch-Baumert K, Mueller-Eckhardt G, Breidenbach M, Kugel HG, et al. The clinical significance of platelet-associated IgG: a study on 298 patients with various disorders. Br J Haematol. 1980;46(1):123–31.

    Article  CAS  PubMed  Google Scholar 

  28. Kiyomizu K, Kashiwagi H, Nakazawa T, Tadokoro S, Honda S, Kanakura Y, et al. Recognition of highly restricted regions in the β-propeller domain of αIIb by platelet-associated anti-αIIbβ3 autoantibodies in primary immune thrombocytopenia. Blood. 2012;120(7):1499–509.

    Article  CAS  PubMed  Google Scholar 

  29. Kuwana M, Kurata Y, Fujimura K, Fujisawa K, Wada H, Nagasawa T, et al. Preliminary laboratory based diagnostic criteria for immune thrombocytopenic purpura: evaluation by multi-center prospective study. J Thromb Haemost. 2006;4(9):1936–43.

    Article  CAS  PubMed  Google Scholar 

  30. Warner MN, Moore JC, Warkentin TE, Santos AV, Kelton JG. A prospective study of protein-specific assays used to investigate idiopathic thrombocytopenic purpura. Br J Haematol. 1999;104(3):442–7.

    Article  CAS  PubMed  Google Scholar 

  31. Brighton TA, Evans S, Castaldi PA, Chesterman CN, Chong BH. Prospective evaluation of the clinical usefulness of an antigen-specific assay (MAIPA) in idiopathic thrombocytopenic purpura and other immune thrombocytopenias. Blood. 1996;88(1):194–201.

    CAS  PubMed  Google Scholar 

  32. 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(3):485–91.

    Article  CAS  PubMed  Google Scholar 

  33. Saito C, Ishiyama K, Yamazaki H, Zaimoku Y, Nakao S. Hypomegakaryocytic thrombocytopenia (HMT): an immune-mediated bone marrow failure characterized by an increased number of PNH-phenotype cells and high plasma thrombopoietin levels. Br J Haematol. 2016;175(2):246–51.

    Article  CAS  PubMed  Google Scholar 

  34. Kaushansky K. The molecular mechanisms that control thrombopoiesis. J Clin Invest. 2005;115(12):3339–47.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Grozovsky R, Begonja AJ, Liu K, Visner G, Hartwig JH, Falet H, et al. The Ashwell-Morell receptor regulates hepatic thrombopoietin production via JAK2-STAT3 signaling. Nat Med. 2015;21(1):47–54.

    Article  CAS  PubMed  Google Scholar 

  36. Kurata Y, Hayashi S, Kiyoi T, Kosugi S, Kashiwagi H, Honda S, et al. Diagnostic value of tests for reticulated platelets, plasma glycocalicin, and thrombopoietin levels for discriminating between hyperdestructive and hypoplastic thrombocytopenia. Am J Clin Pathol. 2001;115(5):656–64.

    Article  CAS  PubMed  Google Scholar 

  37. Kienast J, Schmitz G. Flow cytometric analysis of thiazole orange uptake by platelets: a diagnostic aid in the evaluation of thrombocytopenic disorders. Blood. 1990;75(1):116–21.

    CAS  PubMed  Google Scholar 

  38. Richards EM, Baglin TP. Quantitation of reticulated platelets: methodology and clinical application. Br J Haematol. 1995;91(2):445–51.

    Article  CAS  PubMed  Google Scholar 

  39. Sakuragi M, Hayashi S, Maruyama M, Kabutomori O, Kiyokawa T, Nagamine K, et al. Clinical significance of IPF% or RP% measurement in distinguishing primary immune thrombocytopenia from aplastic thrombocytopenic disorders. Int J Hematol. 2015;101(4):369–75.

    Article  CAS  PubMed  Google Scholar 

Download references

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.

Author information

Authors and Affiliations

  1. Department of Hematology and Oncology, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan

    Hirokazu Kashiwagi M.D.

  2. Department of Blood Transfusion, Osaka University Hospital, 2-15 Yamadaoka, Suita, Osaka, 565-0871, Japan

    Yoshiaki Tomiyama M.D.

Authors
  1. Hirokazu Kashiwagi M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yoshiaki Tomiyama M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hirokazu Kashiwagi M.D. .

Editor information

Editors and Affiliations

  1. Division of Hematology and Oncology Department of Internal Medicine, Iwate Medical University School of Medicine, Morioka, Iwate, Japan

    Yoji Ishida

  2. Department of Blood Transfusion, Osaka University Hospital, Suita, Osaka, Japan

    Yoshiaki Tomiyama

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer Nature Singapore Pte Ltd.

About this chapter

Cite this chapter

Kashiwagi, H., Tomiyama, Y. (2017). Diagnosis in General. In: Ishida, Y., Tomiyama, Y. (eds) Autoimmune Thrombocytopenia　. Springer, Singapore. https://doi.org/10.1007/978-981-10-4142-6_8

Download citation

  • DOI: https://doi.org/10.1007/978-981-10-4142-6_8

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-10-4141-9

  • Online ISBN: 978-981-10-4142-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation