UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Immune thrombocytopenia (ITP) in children: Clinical features and diagnosis

Author
James B Bussel, MD
Section Editor
Donald H Mahoney, Jr, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Immune thrombocytopenia (ITP) of childhood is characterized by isolated thrombocytopenia (platelet count <100,000/microL with normal white blood cell count and hemoglobin). The cause of ITP remains unknown in most cases, but it can be triggered by a viral infection or other immunologic or environmental trigger [1-3]. ITP was previously known as idiopathic thrombocytopenic purpura or immune thrombocytopenic purpura. The current term Immune ThrombocytoPenia preserves the widely-recognized acronym "ITP," while acknowledging the immune-mediated mechanism of the disorder and that patients may have little or no signs of purpura or bleeding [1].

The clinical features and diagnosis of ITP in children will be reviewed here. The initial treatment and management of chronic ITP in children are discussed separately. (See "Immune thrombocytopenia (ITP) in children: Initial management" and "Immune thrombocytopenia (ITP) in children: Management of chronic disease".)

TERMINOLOGY

The following terms are used throughout this topic:

Primary ITP – ITP in the absence of other causes or disorders that may be associated with the thrombocytopenia is known as primary ITP, and is the main focus of this topic review.

Secondary ITP – Secondary ITP refers to immune-mediated thrombocytopenia with an underlying cause, including drug-induced, or associated with systemic illness (eg, systemic lupus erythematosus, HIV). Secondary causes of immune-mediated thrombocytopenia are outlined briefly below. (See 'Differential diagnosis' below.)

                       
To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Oct 2017. | This topic last updated: Oct 30, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
References
Top
  1. Rodeghiero F, Stasi R, Gernsheimer T, 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.
  2. Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010; 115:168.
  3. D'Orazio JA, Neely J, Farhoudi N. ITP in children: pathophysiology and current treatment approaches. J Pediatr Hematol Oncol 2013; 35:1.
  4. 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; 120:574.
  5. Zufferey A, Kapur R, Semple JW. Pathogenesis and Therapeutic Mechanisms in  Immune Thrombocytopenia (ITP). J Clin Med 2017; 6.
  6. Terrell DR, Beebe LA, Vesely SK, et al. The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports. Am J Hematol 2010; 85:174.
  7. Zeller B, Rajantie J, Hedlund-Treutiger I, et al. Childhood idiopathic thrombocytopenic purpura in the Nordic countries: epidemiology and predictors of chronic disease. Acta Paediatr 2005; 94:178.
  8. Kühne T, Buchanan GR, Zimmerman S, et al. 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.
  9. Kühne T, Imbach P, Bolton-Maggs PH, et al. Newly diagnosed idiopathic thrombocytopenic purpura in childhood: an observational study. Lancet 2001; 358:2122.
  10. Chiang MR, Wei CC, Muo CS, et al. Association of primary immune thrombocytopenia and common allergic diseases among children. Pediatr Res 2015; 77:597.
  11. O'Leary ST, Glanz JM, McClure DL, et al. The risk of immune thrombocytopenic purpura after vaccination in children and adolescents. Pediatrics 2012; 129:248.
  12. Kühne T, Berchtold W, Michaels LA, et al. Newly diagnosed immune thrombocytopenia in children and adults: a comparative prospective observational registry of the Intercontinental Cooperative Immune Thrombocytopenia Study Group. Haematologica 2011; 96:1831.
  13. Neunert C, Noroozi N, Norman G, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost 2015; 13:457.
  14. Neunert CE, Buchanan GR, Imbach P, et al. Severe hemorrhage in children with newly diagnosed immune thrombocytopenic purpura. Blood 2008; 112:4003.
  15. Rosthøj S, Hedlund-Treutiger I, Rajantie J, et al. Duration and morbidity of newly diagnosed idiopathic thrombocytopenic purpura in children: A prospective Nordic study of an unselected cohort. J Pediatr 2003; 143:302.
  16. Lilleyman JS. Intracranial haemorrhage in idiopathic thrombocytopenic purpura. Paediatric Haematology Forum of the British Society for Haematology. Arch Dis Child 1994; 71:251.
  17. Psaila B, Petrovic A, Page LK, et al. Intracranial hemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases. Blood 2009; 114:4777.
  18. Medeiros D, Buchanan GR. Current controversies in the management of idiopathic thrombocytopenic purpura during childhood. Pediatr Clin North Am 1996; 43:757.
  19. Kurtzberg J, Stockman JA 3rd. Idiopathic autoimmune thrombocytopenic purpura. Adv Pediatr 1994; 41:111.
  20. Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 2011; 117:4190.
  21. Vesely S, Buchanan GR, Cohen A, et al. Self-reported diagnostic and management strategies in childhood idiopathic thrombocytopenic purpura: results of a survey of practicing pediatric hematology/oncology specialists. J Pediatr Hematol Oncol 2000; 22:55.
  22. Calpin C, Dick P, Poon A, Feldman W. Is bone marrow aspiration needed in acute childhood idiopathic thrombocytopenic purpura to rule out leukemia? Arch Pediatr Adolesc Med 1998; 152:345.
  23. Halperin DS, Doyle JJ. Is bone marrow examination justified in idiopathic thrombocytopenic purpura? Am J Dis Child 1988; 142:508.
  24. Strauss G, Vollert C, von Stackelberg A, et al. Immature platelet count: a simple parameter for distinguishing thrombocytopenia in pediatric acute lymphocytic leukemia from immune thrombocytopenia. Pediatr Blood Cancer 2011; 57:641.
  25. Adly AA, Ragab IA, Ismail EA, Farahat MM. Evaluation of the immature platelet fraction in the diagnosis and prognosis of childhood immune thrombocytopenia. Platelets 2015; 26:645.
  26. McDonnell A, Bride KL, Lim D, et al. Utility of the immature platelet fraction in pediatric immune thrombocytopenia: Differentiating from bone marrow failure and predicting bleeding risk. Pediatr Blood Cancer 2017.
  27. Cybulska A, Meintker L, Ringwald J, Krause SW. Measurements of immature platelets with haematology analysers are of limited value to separate immune thrombocytopenia from bone marrow failure. Br J Haematol 2017; 177:612.
  28. Psaila B, Bussel JB, Frelinger AL, Michelson AD. Differences in platelet function in patients with acute myeloid leukemia and myelodysplasia compared to equally thrombocytopenic patients with immune thrombocytopenia: a reply to a rebuttal. J Thromb Haemost 2013; 11:1002.
  29. Greene LA, Chen S, Seery C, et al. Beyond the platelet count: immature platelet fraction and thromboelastometry correlate with bleeding in patients with immune thrombocytopenia. Br J Haematol 2014; 166:592.
  30. Chong BH. Diagnosis, treatment and pathophysiology of autoimmune thrombocytopenias. Crit Rev Oncol Hematol 1995; 20:271.
  31. Heikal NM, Smock KJ. Laboratory testing for platelet antibodies. Am J Hematol 2013; 88:818.
  32. Sadowitz D, Souid AK, Terndrup TE. Idiopathic thrombocytopenic purpura in children: recognition and management. Pediatr Emerg Care 1996; 12:222.
  33. DiMaggio D, Anderson A, Bussel JB. Cytomegalovirus can make immune thrombocytopenic purpura refractory. Br J Haematol 2009; 146:104.
  34. Teachey DT, Manno CS, Axsom KM, et al. Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). Blood 2005; 105:2443.
  35. Seif AE, Manno CS, Sheen C, et al. Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. Blood 2010; 115:2142.
  36. Wang J, Cunningham-Rundles C. Treatment and outcome of autoimmune hematologic disease in common variable immunodeficiency (CVID). J Autoimmun 2005; 25:57.