Newly Diagnosed Immune Thrombocytopenia: Update on Diagnosis and Management

Abstract

Immune thrombocytopenia (ITP) continues to intrigue pediatricians and hematologists alike. Patients can have a dramatic presentation with wide-spread bleeds over a few days. There is an aura and fear of intra-cranial hemorrhage that drives the physician to recommend and the patient’s family to accept drug treatment. Difference of opinion among physicians in the recommendations for treatment is not uncommon, even though recent evidence-based guidelines recommend a conservative, observation-based approach for the majority of patients with newly diagnosed childhood ITP. It is important to note that a specific ‘platelet cut-off count’, is no longer suggested as an indication by itself to recommend drug therapy. The manuscript is an update on newly diagnosed ITP in children. Recent changes in definitions and recommendations for treatment are highlighted. Pros and cons of 1st line drugs, including corticosteroids, intravenous immunoglobulin and anti-D are listed. Adjunctive therapies for the management of epistaxis and menorrhagia are described. Role of splenic artery embolization and emergency splenectomy in the backdrop of severe thrombocytopenia is discussed. Realistic case scenarios, common errors and frequently asked questions are included for a practical and easy reading.

This is a preview of subscription content, access via your institution.

Fig. 1

References

  1. 1.

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

    PubMed  Article  CAS  Google Scholar 

  2. 2.

    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.

    PubMed  Article  CAS  Google Scholar 

  3. 3.

    Bansal D, Bhamare TA, Trehan A, Ahluwalia J, Varma N, Marwaha RK. Outcome of chronic idiopathic thrombocytopenic purpura in children. Pediatr Blood Cancer. 2010;54:403–7.

    PubMed  Article  Google Scholar 

  4. 4.

    Blanchette V, Bolton-Maggs P. Childhood immune thrombocytopenic purpura: Diagnosis and management. Hematol Oncol Clin North Am. 2010;24:249–73.

    PubMed  Article  Google Scholar 

  5. 5.

    O’Leary ST, Glanz JM, McClure DL, Akhtar A, Daley MF, Nakasato C, et al. The risk of immune thrombocytopenic purpura after vaccination in children and adolescents. Pediatrics. 2012;129:248–55.

    PubMed  Article  Google Scholar 

  6. 6.

    Bansal D, Oberoi S, Marwaha RK, Singhi SC. Approach to a child with bleeding in the emergency room. Indian J Pediatr. 2013;80:411–20.

    PubMed  Article  Google Scholar 

  7. 7.

    Kühne T, Buchanan GR, Zimmerman S, Michaels LA, Kohan R, Berchtold W, et al; Intercontinental Childhood ITP Study Group. A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the Intercontinental Childhood ITP Study group. J Pediatr. 2003;143:605–8.

    PubMed  Article  Google Scholar 

  8. 8.

    Kühne T, Imbach P, Bolton-Maggs PHB, Berchtold W, Blanchette V, Buchanan GR. Intercontinental Childhood ITP Study Group. Newly diagnosed idiopathic thrombocytopenic purpura in childhood: An observational study. Lancet. 2001;358:2122–5.

    PubMed  Article  Google Scholar 

  9. 9.

    Wilson DB. Acquired platelet defects. In: Nathan DG, Orkin SH, Ginsburg D, Look AT, eds. Nathan and Oski’s Hematology of Infancy and Childhood. 7th ed. Philadelphia: WB Saunders Company; 2009. pp. 1557–66.

    Google Scholar 

  10. 10.

    Lileyman JS. On behalf of the pediatric hematology forum of the British society of hematology. Intracranial hemorrhage in idiopathic thrombocytopenic purpura. Arch Dis Child. 1994;72:251–3.

    Article  Google Scholar 

  11. 11.

    Oberoi S. Bansal D. ABC of CBC. In: Hooja S, Aggarwal A, Aggarwal A, eds. Jaipur: Souvenir Pedicon; 2011. pp. 24–7.

  12. 12.

    The Indian Society of Hematology and Blood Transfusion (ISHBT) Consensus Guidelines for Immune Thrombocytopenia. Nov 2011. Version 1.0. Available from: http://ishbt.org/wp-content/uploads/guide.pdf.

  13. 13.

    Bansal D. Childhood chronic ITP. Pediatr Blood Cancer. 2011;56:875.

    PubMed  Article  Google Scholar 

  14. 14.

    Khunhausen S, Nichols DJ, Noonan N, et al. Epistaxis Procedure, Children’s Hospital Oakland Department of Nursing. Poster presented at: Association of Pediatric Oncology Nurses Conference Philadelphia, 2003; Philadelphia.

  15. 15.

    Agrawal AK, Hastings CA, Feusner J. Hematologic supportive care for children with cancer. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia: Williams & Wilkins; 2011. pp. 1167–8.

    Google Scholar 

  16. 16.

    Biggs TC, Nightingale K, Patel NN, Salib RJ. Should prophylactic antibiotics be used routinely in epistaxis patients with nasal packs? Ann R Coll Surg Engl. 2013;95:40–2.

    PubMed  Article  CAS  PubMed Central  Google Scholar 

  17. 17.

    Qureishi A, Burton MJ. Interventions for recurrent idiopathic epistaxis (nosebleeds) in children. Cochrane Database Syst Rev. 2012;9:CD004461.

  18. 18.

    Rodeghiero F. Management of menorrhagia in women with inherited bleeding disorders: General principles and use of desmopressin. Haemophilia. 2008;14:S21–30.

    Article  Google Scholar 

  19. 19.

    Ahuja SP, Hertweck SP. Overview of bleeding disorders in adolescent females with menorrhagia. J Pediatr Adolesc Gynecol. 2010;23:S15–21.

    PubMed  Article  Google Scholar 

  20. 20.

    Ramesh Kumar R, Singhi SC, Singhi P. Raised Intracranial Pressure (ICP): Management in emergency department. Indian J Pediatr. 2012;79:518–24.

    PubMed  Article  CAS  Google Scholar 

  21. 21.

    Madoff DC, Denys A, Wallace MJ, Murthy R, Gupta S, Pillsbury EP, et al. Splenic arterial interventions: Anatomy, indications, technical considerations, and potential complications. Radiographics. 2005;25:S191–211.

    PubMed  Article  Google Scholar 

  22. 22.

    Puapong D, Terasaki K, Lacerna M, Applebaum H. Splenic artery embolization in the management of an acute immune thrombocytopenic purpura related intracranial hemorrhage. J Pediatr Surg. 2005;40:869–71.

    PubMed  Article  Google Scholar 

  23. 23.

    Wu Z, Zhou J, Pankaj P, Peng B. Laparoscopic splenectomy for immune thrombocytopenia (ITP) patients with platelet counts lower than 1×109/L. Int J Hematol. 2011;94:533–8.

    PubMed  Article  Google Scholar 

  24. 24.

    Sharma SK, Gupta N, Seth T, Srinivas M, Mishra P, Mahapatra M. Successful management of refractory chronic immune thrombocytopenia with intracranial hemorrhage by emergency splenectomy. Indian J Pediatr. 2012;79:397–8.

    PubMed  Article  Google Scholar 

  25. 25.

    Barnes C, Blanchette V, Canningyand P, Carcao M. Recombinant FVIIa in the management of intracerebral haemorrhage in severe thrombocytopenia unresponsive to platelet-enhancing treatment. Transfus Med. 2005;15:145–50.

    PubMed  Article  CAS  Google Scholar 

  26. 26.

    Kime C, Klima J, Rose MJ, O’Brien SH. Patterns of inpatient care for newly diagnosed immune thrombocytopenia in US children’s hospitals. Pediatrics. 2013;131:880–5.

    PubMed  Article  Google Scholar 

  27. 27.

    Grainger JD, Rees JL, Reeves M, Bolton-Maggs PHB. Changing trends in the UK management of childhood ITP. Arch Dis Child. 2012;97:8–11.

    PubMed  Article  Google Scholar 

  28. 28.

    Psaila B, Petrovic A, Page LK, Menell J, Schonholz M, Bussel JB. Intracranial hemorrhage in children with immune thrombocytopenia: Study of 40 cases. Blood. 2009;114:4777–83.

    PubMed  Article  CAS  PubMed Central  Google Scholar 

  29. 29.

    Buchanan GR. Bleeding signs in children with idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol. 2003;25:S42–6.

    PubMed  Article  Google Scholar 

  30. 30.

    Yewale V, Choudhury P, Thacker N, eds. IAP guide book on immunization: Immunization in special circumstances, 2010. pp. 143–50. Available from: http://www.iapcoi.com/hp/iap_guidebook.php

  31. 31.

    Meyer O, Kiesewetter H, Hermsen M, Salama A. Efficacy and safety of anti-D given by subcutaneous injection to patients with autoimmune thrombocytopenia. Eur J Haematol. 2004;73:71–2.

    PubMed  Article  CAS  Google Scholar 

  32. 32.

    Trebo MM, Frey E, Gadner H, Minkov M. Subcutaneous anti-D globulin application is a safe treatment option of immune thrombocytopenia in children. Ann Hematol. 2010;89:415–8.

    PubMed  Article  CAS  Google Scholar 

  33. 33.

    D’Orazio JA, Neely J, Farhoudi N. ITP in children: Pathophysiology and current treatment approaches. J Pediatr Hematol Oncol. 2013;35:1–13.

    PubMed  Article  Google Scholar 

  34. 34.

    Rajendran A, Bansal D. ITP: The dilemma of treatment. J Pediatr Hematol Oncol. 2013. [Epub ahead of print]

Download references

Conflict of Interest

None.

Role of Funding Source

None.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Sunit Singhi.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Bansal, D., Rajendran, A. & Singhi, S. Newly Diagnosed Immune Thrombocytopenia: Update on Diagnosis and Management. Indian J Pediatr 81, 1033–1041 (2014). https://doi.org/10.1007/s12098-013-1217-2

Download citation

Keywords

  • Acute ITP
  • Bone marrow
  • Idiopathic thrombocytopenic purpura
  • Tranexamic acid