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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 count (FBC) and film
    2. Alternate causes for petechiae and purpura need to be excluded
    3. The decision to treat a child should be based on clinical symptoms and not the platelet count; the majority do not require treatment
    4. The risk of intracranial haemorrhage in ITP is very low (<1%)

    Background

    ITP is an autoimmune bleeding disorder characterised by all three of:

    1. Isolated thrombocytopenia (platelet count of <100 x 109/L, often < 20 x 109/L)
    2. Well child with no concerning features on clinical history or examination
    3. Otherwise normal FBC and film

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

    Newly diagnosed ITP is within 3 months of diagnosis. ITP often resolves within 3 months, and resolves in 75% of children by 6 months. Chronic ITP is longer than 12 months

    Assessment

    History and examination

    • Sudden onset petechial rash or bruising
    • Bleeding symptoms can also include:
      • epistaxis, gum or gastrointestinal bleeding, haematuria or menorrhagia
      • very rarely intracranial haemorrhage (ICH): headache, nausea, vomiting, lethargy, irritability, decreased consciousness or neurological symptoms
    • Preceding viral infection or recent live virus immunisation e.g. MMR
    • Well looking with normal clinical observations

    Red flags for alternate 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
    • Non-accidental injury
    • Aplastic anaemia
    • Systemic lupus erythematosus
    • Drug-induced thrombocytopaenia
    • Thrombotic thrombocytopenic purpura or haemolytic uraemic syndrome

    Investigations

    • FBC and blood film is the only initial investigation required
    • Film must be reviewed to exclude an alternate diagnosis

    Management

    The decision to treat a child should be based on the clinical symptoms and not the platelet count. Treatment decisions also need to take into consideration the presence of active bleeding, the risk of future bleeding (e.g. impending surgery) and psychosocial factors

    Risk category

    Symptoms

    Management

    Low

    Many petechiae or large bruises
    Painless oral/palatal petechiae or purpura
    Blood crusting in nares

    Outpatient without medical treatment (Unless significant psychosocial or safety concerns)
    Repeat FBC and review in 1 week
    Provide family education

    Moderate

    Epistaxis > 5 mins
    Haematuria
    Haematochezia
    Painful oral purpura
    Significant menorrhagia

    Often require hospital admission
    Film must be reviewed by a haematologist prior to starting treatment 
    Increase platelet count to stop bleeding (not to normal level)
    First line: oral prednisolone 2mg/kg (max 60mg) for 4-7 days
    Second line if poor response or rapid platelet rise is required (e.g. prior to surgery): IVIG 0.8 -1g/kg (discuss with haematology team)
    Additional treatments:

    Severe

    Suspected internal haemorrhage (brain, lung, muscle, joint, etc) OR mucosal bleeding that requires immediate intervention

    Urgent consultation with haematology team
    Combination IVIG 0.8-1g/kg and pulse IV methylprednisolone 15-30mg/kg (max 1g) daily for 3 days
    Platelet transfusion 20ml/kg, continuous if required
    IV tranexamic acid 15mg/kg
    Urgent surgical intervention or referral depending on site of bleeding

    Life-threatening

    Documented ICH or life-threatening bleeding at any site

    Consult haematology team for:

    • Severe or life-threatening bleeding
    • < 1 year or > 14 years of age
    • Any abnormality on blood film
    • Any concern for possible alternate 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 (e.g. footy, rugby, soccer, hockey and martial arts)
      • Limit activities that have a risk for traumatic injury (e.g. horse-riding, riding a scooter, skate-board or bike, climbing on play-ground equipment)
    • Avoid anti-platelet, non-steroidal and anticoagulant medications. Avoid intramuscular injections
    • Monitor for significant bleeding symptoms and go immediately to the emergency department if they occur
    • Monitor for signs of ICH and go immediately to the emergency department if head injury or severe headache

    Consider consultation with local paediatric team when

    • Uncertainty about diagnosis, any red flags, or to arrange follow up
    • Significant concern about the family’s ability to enact the management plan or attend follow-up

    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

    Parent information

    Idiopathic thrombocytopenic purpura

    Last updated March 2020

  • 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' Blood132(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)