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Immune Thrombocytopenic Purpura

Immune Thrombocytopenic Purpura is a topic covered in the 5-Minute Pediatric Consult.

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Basics

Description

  • Immune thrombocytopenic purpura (ITP) is an autoimmune syndrome characterized by the following:
    • Isolated thrombocytopenia (platelet count formally <100,000/mm3, typically <20,000/mm3)
    • Shortened platelet survival
    • Increased number of megakaryocytes in the bone marrow
  • Primary ITP implies absence of other causes for thrombocytopenia; it often follows a viral infection.
  • Secondary ITP indicates other immune dysregulation associated with thrombocytopenia.
  • Phases of ITP
    • Newly diagnosed (acute) ITP: within 3 months of initial diagnosis
    • Persistent ITP: transient improvement or continued thrombocytopenia for 3 to 12 months
    • Chronic ITP: persistent thrombocytopenia >12 months after initial presentation

Epidemiology

  • Most common acquired platelet disorder in childhood
  • Incidence is 5/100,000 children per year (<15 years of age).
  • Often follows viral syndrome by a few weeks; this may be associated with higher likelihood of spontaneous recovery.
  • Males and females are equally affected in childhood ITP but mild male predominance in younger children
  • Female-to-male ratio is 3:1 in adult and chronic ITP.
  • Median age at diagnosis is 4 years. Children <1 year or >10 years are more likely to develop chronic ITP.
  • >70% of childhood ITP resolves within 6 months.
  • Risk of severe bleeding is <5% and of intracranial bleeding is <0.5%.

Pathophysiology

  • Thrombocytopenia due to increased destruction of antibody-coated platelets in the reticuloendothelial system, particularly the spleen
  • Hypothesized that antibodies generated in response to foreign antigen or drug cross-react with platelet membrane glycoproteins (most commonly IIb/IIIa and Ib/IX)
  • Other mechanisms of immune dysregulation have been implicated, including possible inhibition of thrombocytopoiesis, limiting ability to compensate for destruction.
  • Typical bone marrow aspirate shows increased numbers of immature megakaryocytes.

Commonly Associated Conditions

  • In younger children, primary ITP is the most common presentation of ITP.
  • Secondary ITP is seen with autoimmune disorders (e.g., systemic lupus erythematosus [SLE], autoimmune lymphoproliferative syndrome [ALPS]).
  • HIV

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Citation

Cabana, Michael D., editor. "Immune Thrombocytopenic Purpura." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618188/all/Immune_Thrombocytopenic_Purpura.
Immune Thrombocytopenic Purpura. In: Cabana MD, ed. 5-Minute Pediatric Consult. 8th ed. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618188/all/Immune_Thrombocytopenic_Purpura. Accessed April 21, 2019.
Immune Thrombocytopenic Purpura. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult. Available from https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618188/all/Immune_Thrombocytopenic_Purpura
Immune Thrombocytopenic Purpura [Internet]. In: Cabana MD, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2019 April 21]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618188/all/Immune_Thrombocytopenic_Purpura.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Immune Thrombocytopenic Purpura ID - 618188 ED - Cabana,Michael D, BT - 5-Minute Pediatric Consult UR - https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618188/all/Immune_Thrombocytopenic_Purpura PB - Wolters Kluwer ET - 8 DB - Pediatrics Central DP - Unbound Medicine ER -