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Clinical Practice Guidelines

Immune thrombocytopenic purpura

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  • Immune thrombocytopenic purpura

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    See also

    Fever and petechiae - purpura

    Key points

    1. Immune thrombocytopenia (ITP) is an isolated low platelet count of <100 x 109/L in a well child with an otherwise normal full blood examination (FBE) and film
    2. Alternative causes for petechiae and purpura need to be excluded
    3. The decision to treat a child should be based on clinical features and not the platelet count; the majority do not require treatment
    4. The risk of intracranial haemorrhage (ICH) in ITP is very low (<1%)

    Background

    ITP is an autoimmune bleeding disorder characterised by all three of

    • Isolated thrombocytopenia (platelet count <100 x 109/L)
    • Well child with no concerning features on clinical history or examination
    • Otherwise normal FBE and blood film

    Although relatively uncommon, ITP is the most common cause of symptomatic acquired thrombocytopenia in children. It is a diagnosis of exclusion as there is no specific laboratory test to confirm the diagnosis

    • Newly diagnosed ITP
      • most common in young children (2-6 years)
      • typically self-limiting
      • often follows a viral infection
      • typically presents with sudden onset of symptoms
      • spontaneous recovery within 3-6 months of diagnosis
    • Persistent ITP -- thrombocytopenia lasting 3-12 months from time of diagnosis
    • Chronic ITP -- persistent thrombocytopenia >12 months. More common in adolescents (20-30% of adolescents)

    Assessment

    History and examination

    • Sudden onset petechial rash; easy bruising in an otherwise often well looking child
    • Excessive bleeding or bruising with injuries
    • Epistaxis, gingival or gastrointestinal bleeding, haematuria or menorrhagia
    • ICH (rare <1%): headache, nausea, vomiting, lethargy, irritability, decreased consciousness or neurological symptoms
    • Preceding viral infection (eg CMV, EBV, VZV) or recent live virus immunisation (eg MMR)
    • Recent changes in medication
    • Family history of autoimmune condition(s)

    Red flags for alternative diagnosis

    • Bone pain, limp, anorexia, weight loss, jaundice, fever, sweats or infective symptoms
    • Rash, arthritis, myalgias, dry eyes, mouth ulcers, recurrent infections or fever
    • Family or personal history of bleeding disorders
    • Recent medication use (NSAIDs, anticoagulants or other anti-platelet medications)

    Differential diagnoses

    • Leukaemia
    • Bacterial or viral infection
    • Inflicted injury
    • Bone marrow disorders eg aplastic anaemia
    • Systemic lupus erythematosus
    • Drug-induced thrombocytopenia
    • Thrombotic thrombocytopenic purpura or haemolytic uraemic syndrome
    • Localised petechiae in an SVC distribution following vomiting/valsalva

    Assessment of severity

    Modified Buchanan bleeding score

    Bleeding Grade Bleeding risk Description
    0 None No new haemorrhage
    1 Minor Few petechiae (≤100) and/or ≤5 small bruises (≤3 cm diameter), no mucosal bleeding
    2 Mild Petechiae (>100) and/or >5 bruises (>3 cm diameter)
    3a Moderate
    Low risk    		 Blood in nares, painless oral purpura, oral/palatal petechiae, buccal purpura along molars only, epistaxis ≤5 mins
    3b Moderate high risk Epistaxis >5 min, haematuria, haematochezia, painful oral purpura, significant menorrhagia
    4 Severe Mucosal bleeding or suspected internal haemorrhage (brain, lung, joint, muscle etc)
    5 Life-threatening Documented ICH or life-threatening bleeding of any site

    Management

    Investigations

    • FBE and blood film is the only initial investigation required
    • Film must be reviewed to exclude an alternative diagnosis
    • Other investigations should be targeted based on likely differential diagnosis or any complications

    Treatment

    • The decision to treat a child should be based on clinical features and not platelet count
    • Treatment decisions also need to take into consideration the presence of active bleeding, the risk of future bleeding (eg impending surgery) and psychosocial factors
    • The goal of treatment is to stop active bleeding, not to normalise platelet count
    Risk category
    (Based on modified Buchanan bleeding score)    		 Symptoms Management
    Low
    (0-3a)    		 Petechiae/bruising
    Painless oral/palatal petechiae or purpura
    Blood crusting in nares    		 Outpatient without medical treatment (unless significant psychosocial or safety concerns)
    Repeat FBE and review in 1 week
    Provide family education (see below)
    Moderate
    (3b)    		 Epistaxis >5 mins
    Haematuria
    Haematochezia
    Painful oral purpura
    Significant menorrhagia    		 Often requires hospital admission
    Film must be manually reviewed by or discussed with a haematologist prior to starting treatment
    Increase platelet count to stop bleeding (not to normal level)
    First line: oral prednisolone 2-4 mg/kg (max 100 mg) for 4–7 days

    Second line, if poor response or rapid platelet rise is required (eg prior to surgery): IVIg 0.8–1 g/kg (discuss with Haematology)

    Additional treatments (consider referral to specialty teams as appropriate):
    Severe
    (4)    		 Suspected internal haemorrhage (brain, lung, muscle, joint, etc) OR mucosal bleeding that requires immediate intervention Urgent consultation with Haematology
    Combination IVIg 0.8–1 g/kg and pulse IV methylprednisolone 30 mg/kg (max 1 g) daily for 3 days
    Platelet transfusion 20 mL/kg, continuous if required
    Tranexamic acid 15 mg/kg (max 1-1.5g) IV

    If life threatening, thrombopoietin receptor agonists (e.g. Romiplostin or Eltrombopag)
    Urgent surgical intervention or referral depending on site of bleeding
    Life-threatening
    (5)    		 Documented ICH or life-threatening bleeding at any site

    Consult Haematology for:

    • Severe or life-threatening bleeding
    • <1 year or >14 years of age
    • Any abnormality on blood film
    • Any concern for possible alternative diagnosis
    • Chronic ITP
    • Head injury or signs of ICH

    Provide family education

    • Provide written information and letter to document diagnosis
    • Restrict activities to minimise the risk of head injury:
      • Avoid contact sports eg football, rugby, soccer, hockey and martial arts
      • If any head injury is sustained, patients are advised to present to ED
      • Limit activities with risk for traumatic injury eg horse-riding, skiing, scooter, skateboard or bike riding, climbing on play-ground equipment including trampolines
    • Avoid anti-platelet, non-steroidal and anticoagulant medications
    • Avoid intramuscular injections
    • Monitor for significant bleeding symptoms; requires emergency department review
    • Monitor for signs of ICH; requires emergency department review

    Consider consultation with local paediatric team when

    • Uncertainty about diagnosis, any red flags, or to arrange follow up
    • Significant concern about family's ability to enact management plan or attend follow up
    • Referral for ongoing management: Haematology, General Paediatrics or GP depending on clinical context and local practice

    Consider transfer when

    • Level of care exceeds the comfort of the local health service
    • Severe or life-threatening haemorrhage

    For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services

    Consider discharge when

    Family understands the condition, management, activity restrictions, follow-up plan and when to go to the emergency department

    Follow up plan needs to be specific, including timing of repeat FBE

    Reference list

    1. Cooper N. A review of the management of childhood immune thrombocytopenia: how can we provide an evidence-based approach? Br J Haematol. 2014. 165(6), p756-67.
    2. Grace, RF et al. An update on Paediatric ITP: differentiating primary ITP, IPD, and PID. Blood. 2022. 140(6), p542-555.
    3. Kim TO, Despotovic JM. Pediatric immune thrombocytopenia (ITP) treatment. Annals of Blood. 2021. 6, p9.
    4. Lee JM. Advances in management of pediatric chronic immune thrombocytopenia: a narrative review. J Yeungnam Med Sci. 2023. 40(3), p241-246.
    5. Livingston, J et al. Evaluating the impact of Thrombopoietin Receptor Agonist medications on patient outcomes and quality of life in Pediatric Immune Thrombocytopenia. Blood. 2021. 138(supplement 1), p4072.
    6. Matzdorff, A et al. Immune Thrombocytopenia - Current Diagnostics and Therapy: Recommendations of a Joint Working Group of DGHO, ÖGHO, SGH, GPOH, and DGTI. Oncol Res Treat. 2018. 41 Suppl 5, p1-30.
    7. Neunert, C et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Advances. 2019. 3(23), p3829-3866.
    8. Rodeghiero, F 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, p2386–2393.
    9. Thakur, Y et al. Diagnosis and Management of Immune Thrombocytopenia in Paediatrics: A Comprehensive Review. Cureus. 2024. 16(9).
    10. Verissimo, V et al. Australian and New Zealand consensus guideline for paediatric newly diagnosed immune thrombocytopaenia endorsed by Australian New Zealand Children's Haematology and Oncology Group. J Paediatr Child Health. 2023. 59(5), p711-717.

    Last updated April 2025

  • Reference List

    1. D'Orazio JA et al, 2013, 'ITP in children: pathophysiology and current treatment approaches' Journal of pediatric hematology and oncology
    2. Friedman JN et al, 2018, 'Canadian Paediatric Society, Diagnosis and management of typical newly diagnosed primary immune thrombocytopenia (ITP) in childhood' viewed 19 March 2020 <https://www.cps.ca/en/documents/position/immune-thrombocytopenia>
    3. Grainger JD, 2015, ‘North-west guideline for the management of child with suspected ITP’ viewed 19 March 2020, <http://www.uk-itp.org/docs/ITP/suspected_or_known_immune_thrombocytopenia_management_plan__children_.pdf>
    4. Heitink-Pollé KM, 2018, 'Intravenous immunoglobulin vs observation in childhood immune thrombocytopenia: a randomized controlled trial' Blood, 132(9), 883-891.
    5. Kühne T et al, 2003 ‘A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the intercontinental childhood ITP study group’ The Journal of Pediatrics, 143(5):605-8.
    6. Neunert C et al, 2011, 'The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia' Blood 117(16): 4190-4207.
    7. Neunert C et al, 2015, 'Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review' Journal of thrombosis and haemostasis 13(3): 457-464.
    8. Psaila B et al, 2009, ‘Intracranial haemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases’ Blood, Nov 26; 114 (23); 4777 – 4783
    9. Queensland Children’s Hospital, 2019, ‘Newly Diagnosed Immune Thrombocytopaenia Purpura Guideline’ viewed 19 March 2020 <https://www.childrens.health.qld.gov.au/wp-content/uploads/PDF/guidelines/gdl-02923.pdf>
    10. Rodeghiero F et al, 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): 2386-2393.
    11. Schoettler ML et al, 2017, ‘Increasing observation rates in low-risk pediatric immune thrombocytopenia using a standardized clinical assessment and management plan (SCAMP(R))’ Pediatric blood & cancer, 2017;64(5)