Immune thrombocytopenic purpura

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

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

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