📋 Key Information Summary
- Immune thrombocytopenia (ITP) is an autoimmune condition characterised by isolated thrombocytopenia (platelets <100×10⁹/L) in the absence of another identifiable cause — it remains a diagnosis of exclusion.
- In adults, ITP is typically chronic with insidious onset; in children it is usually acute and self-limiting following a viral illness, resolving within 6 months in >80%.
- A peripheral blood film must be reviewed to exclude pseudothrombocytopenia, spurious counts, schistocytes (TTP/HUS), or leukaemic blasts before diagnosing ITP.
- Bone marrow biopsy is NOT routinely required in typical presentations but should be considered before second-line therapy or if there are atypical features (age >60, refractory disease, or abnormal blood film).
- Observation without treatment is appropriate if platelets >30×10⁹/L AND there is no clinically significant bleeding, regardless of patient age.
- First-line treatment for significant bleeding or counts <30×10⁹/L with bleeding risk includes corticosteroids (prednisolone 1 mg/kg/day PO for 1–2 weeks then taper) or high-dose dexamethasone (40 mg/day PO for 4 days).
- IV immunoglobulin (IVIG 1 g/kg) or anti-D immunoglobulin (WinRho® 50 µg/kg IV, Rh-D positive non-splenectomised patients only) provide rapid platelet response in emergencies or pre-procedural settings.
- Tranexamic acid (1 g PO/IV TDS) is a useful adjunct for mucosal bleeding; avoid NSAIDs and antiplatelet agents in all ITP patients.
- Avoid intramuscular injections, rectal examinations, and contact sports in patients with platelets <50×10⁹/L; counsel on trauma and bleeding risk.
- Splenectomy remains a durable second-line option with ~60–70% long-term response; thrombopoietin receptor agonists (romiplostim, eltrombopag) and rituximab are increasingly used as second-line alternatives.
- All patients should receive pneumococcal, Haemophilus influenzae type b, and meningococcal vaccinations at least 2 weeks before elective splenectomy.
- Aboriginal and Torres Strait Islander Australians may present later with more severe disease; ensure culturally safe communication, appropriate health-literacy resources, and link with Indigenous health services.
- Urgent haematology referral is recommended for all new suspected ITP cases, platelets <20×10⁹/L, active clinically significant bleeding, or failure of first-line therapy.
- Emergency treatment with IVIG + corticosteroids ± platelet transfusion is required for life-threatening bleeding (intracranial, gastrointestinal, or genitourinary haemorrhage).
Introduction & Australian Epidemiology
Immune thrombocytopenia (ITP), formerly termed idiopathic thrombocytopenic purpura, is an acquired autoimmune disorder in which antibody- and cell-mediated destruction of platelets, together with impaired megakaryopoiesis, results in isolated thrombocytopenia. The condition affects both children and adults, though the clinical course differs markedly between these populations.
In Australia, the estimated incidence of adult ITP is approximately 3.3 per 100,000 person-years, with a prevalence of 9–12 per 100,000. Paediatric ITP is more common, with an incidence of approximately 5 per 100,000 children per year. The condition shows a bimodal age distribution, with a peak in childhood (2–6 years) and a second peak in adults aged 20–50 years, with a slight female preponderance in adults.
In children, over 80% of cases resolve spontaneously within 6 months (acute ITP). In contrast, adult ITP is more frequently chronic, with approximately 60–70% of patients requiring ongoing treatment beyond 12 months. Mortality in adult ITP is modestly increased compared with the general population (standardised mortality ratio ~1.3–2.0), primarily due to bleeding and infection-related complications.
ITP may occur in isolation (primary ITP) or in association with other conditions including systemic lupus erythematosus (SLE), antiphospholipid syndrome, common variable immunodeficiency (CVID), hepatitis C virus (HCV) infection, Helicobacter pylori infection, HIV, and chronic lymphocytic leukaemia (CLL). Identifying and treating secondary causes is an essential component of management.
Diagnosis of Exclusion
ITP is defined as isolated thrombocytopenia (platelet count <100×10⁹/L) with normal white cell count, haemoglobin (unless bleeding-related), and coagulation profile (PT/APTT/INR normal), in the absence of another identifiable cause. There is no single confirmatory test; the diagnosis is established by excluding alternative aetiologies.
Essential Diagnostic Criteria
- Platelet count <100×10⁹/L on at least two occasions, separated by >24 hours (to exclude transient post-viral thrombocytopenia in children).
- Peripheral blood film reviewed by an experienced haematologist or haematology scientist — excludes pseudothrombocytopenia (EDTA-dependent clumping), dysplasia, schistocytes (thrombotic thrombocytopenic purpura / haemolytic uraemic syndrome), leukaemic blasts, and giant platelets (MYH9-related disorders).
- Full blood count with differential showing no anaemia (unless attributable to bleeding) and normal white cell morphology.
- Coagulation studies (PT, APTT, fibrinogen) within normal limits.
- No palpable splenomegaly on examination (if present, consider hypersplenism, lymphoproliferative disease, or chronic liver disease).
Recommended Baseline Investigations
Differential Diagnosis of Thrombocytopenia
| Category | Condition | Key Distinguishing Features |
|---|---|---|
| Pseudothrombocytopenia | EDTA-dependent platelet clumping | Platelet count normalises on citrate sample; film shows clumps |
| Spurious | Giant platelets counted as RBCs (MYH9 disorders) | Large platelets on film; normal platelet mass |
| Consumptive | TTP / HUS | Schistocytes, anaemia, ↑LDH, neurological or renal features; medical emergency |
| Consumptive | DIC | Prolonged PT/APTT, low fibrinogen, elevated D-dimer |
| Drug-induced | Heparin-induced thrombocytopenia (HIT) | Onset 5–14 days after heparin exposure; thrombosis paradox; PF4 antibody positive |
| Drug-induced | Other drugs (valproate, quinine, sulfonamides, linezolid) | Temporal relationship with drug exposure; resolves on withdrawal |
| Hypersplenism | Chronic liver disease, portal hypertension | Palpable splenomegaly, deranged LFTs, signs of chronic liver disease |
| Bone marrow failure | MDS, aplastic anaemia, leukaemia infiltration | Cytopenias in multiple lineages; dysplasia; abnormal marrow |
| Infection-related | Viral (EBV, CMV, dengue), sepsis | Systemic features; self-limiting if viral; positive cultures if bacterial |
| Pregnancy-specific | Gestational thrombocytopenia, pre-eclampsia/HELLP | Usually late pregnancy; platelets typically >70×10⁹/L in gestational; HELLP has elevated transaminases |
Initial Management Thresholds
The decision to treat ITP depends on the platelet count AND the clinical bleeding status. Many patients with counts above 30×10⁹/L and no bleeding can be safely observed without treatment.
Severity Stratification & Treatment Thresholds
First-Line Pharmacological Therapy
Emergency Management of Life-Threatening Bleeding
- Platelet transfusion (1 adult dose, can repeat) — response will be poor but is necessary in emergency
- IVIG 1 g/kg IV (Intragam® P)
- IV methylprednisolone 1 g/day for 3 days OR dexamethasone 40 mg IV
- Tranexamic acid 1 g IV stat then 1 g IV TDS
- Recombinant factor VIIa (NovoSeven®) — haematology-guided only in refractory cases
- Urgent emergency splenectomy may be required if medical therapy fails
Contact on-call haematology immediately. Arrange ICU admission if haemodynamically unstable or intracranial bleeding suspected.
Second-Line Therapies (for chronic/refractory ITP — Haematologist-directed)
Splenectomy
Laparoscopic splenectomy offers a durable complete response in approximately 60–70% of patients with chronic ITP. It is generally considered after failure of at least one medical second-line agent (TPO-RA or rituximab) or if TPO-RAs and rituximab are not suitable. Key considerations include:
- Vaccinate against Streptococcus pneumoniae (2 vaccines: Prevenar 13® then Pneumovax 23® at least 2 weeks pre-splenectomy), Neisseria meningitidis (Menactra® or Nimenrix® — ACWY + B), and Haemophilus influenzae type b (Hib) at least 2 weeks before surgery.
- Ensure lifelong post-splenectomy antibiotic prophylaxis (phenoxymethylpenicillin 250 mg PO BD or erythromycin 250 mg PO BD if penicillin-allergic) — patient must carry a MedicAlert bracelet.
- Thromboprophylaxis post-operatively (LMWH) as per surgical protocol.
- Risk of overwhelming post-splenectomy infection (OPSI), particularly with encapsulated organisms — annual influenza vaccination and revaccination schedule per ATAGI guidelines.
Monitoring During Treatment
Primary-Care Role
General practitioners play a critical role in the long-term management and supportive care of patients with ITP, even when haematology is directing therapy. The following practical measures are essential in the primary-care setting.
Medications to Avoid
- NSAIDs (ibuprofen, naproxen, diclofenac, celecoxib) — impair platelet function AND increase GI bleeding risk. Use paracetamol for analgesia; if inadequate, discuss with haematology.
- Aspirin and antiplatelet agents (clopidogrel, ticagrelor, prasugrel) — contraindicated unless specifically indicated under haematology guidance (e.g., post-coronary stent with dual-discussion).
- Intramuscular (IM) injections — avoid when platelets <50×10⁹/L due to risk of deep muscle haematoma. Vaccinations should be given subcutaneously where possible.
- Anticoagulants (warfarin, DOACs, heparin) — use with extreme caution and haematology co-management if indicated.
- Rectal examinations and suppositories — avoid when platelets <50×10⁹/L.
Patient Counselling
- Explain that ITP is an autoimmune condition — the immune system is destroying platelets, not cancer.
- Counsel on signs of significant bleeding: persistent nosebleeds (>20 minutes), blood in urine or stools, heavy menstrual bleeding, unexplained bruising spreading rapidly, wet purpura (blood blisters inside the mouth).
- Advise to present immediately to the emergency department if severe headache occurs (risk of intracranial haemorrhage) or if significant trauma occurs.
- Avoid contact sports, activities with high injury risk, and use of sharp objects when platelets are <50×10⁹/L.
- Wear a medical alert identification (bracelet or necklace) if platelets are chronically low or if splenectomised.
- Dental procedures — inform the dentist of ITP status; platelet count >30×10⁹/L is generally safe for dental extractions with local measures (tranexamic acid mouthwash, gelatin sponge). Discuss with haematology for counts <30×10⁹/L.
- Menorrhagia management — refer to gynaecology if heavy menstrual bleeding is impacting quality of life; tranexamic acid and hormonal therapies (combined OCP, LNG-IUS) can be used with haematology guidance.
Pre-Splenectomy Vaccination Protocol
When splenectomy is planned, the following vaccines should be administered at least 2 weeks before surgery. If urgent splenectomy is required, vaccinate as soon as possible post-operatively (but note reduced immunogenicity).
| Vaccine | Product | Schedule | PBS / NIP Status |
|---|---|---|---|
| Pneumococcal conjugate (PCV13) | Prevenar 13® | Single dose SC | Funded for asplenic patients under NIP |
| Pneumococcal polysaccharide (PPV23) | Pneumovax 23® | Single dose SC ≥8 weeks after PCV13, then repeat every 5 years | Funded for asplenic patients under NIP |
| Meningococcal ACWY | Menactra® / Nimenrix® | Single dose SC; booster every 5 years | Funded for asplenic patients under NIP |
| Meningococcal B | Bexsero® | 2 doses SC, ≥4 weeks apart | Not NIP-funded for adults; private purchase (~$130–150/dose) |
| Haemophilus influenzae type b (Hib) | ActHIB® | Single dose SC (if not previously vaccinated as adult) | Funded for asplenic patients under NIP |
| Influenza (annual) | Flu vaccine (quadrivalent) | Annual, before winter season | NIP-funded annually for all Australians |
| COVID-19 | Per current ATAGI recommendations | As per current booster schedule | NIP-funded |
Routine Monitoring in Primary Care (Stable Chronic ITP)
- FBC every 2–3 months if stable and on no active treatment.
- Annual review of bleeding symptoms, quality of life, and medication review.
- Monitor for complications of chronic steroid use (osteoporosis screening with DEXA if >3 months cumulative use; diabetes screening; cataracts; adrenal suppression).
- Bone protection: calcium + vitamin D supplementation; consider bisphosphonate if prolonged steroid use expected.
- Mental health screening — chronic ITP and its treatments are associated with anxiety, depression, and fatigue.
When to Refer
Most patients with newly diagnosed ITP should be referred to haematology for confirmation of diagnosis, risk stratification, and treatment planning. The urgency and level of referral depend on the clinical scenario.
Special Populations
Pregnancy
Paediatrics
Elderly (≥65 years)
Renal Impairment
Hepatic Impairment
Immunocompromised
Aboriginal and Torres Strait Islander Health Considerations
📚 References
- 1. Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Advances. 2019;3(23):3829–3866. doi:10.1182/bloodadvances.2019000966.
- 2. Provan D, Arnold DM, Bussel JB, et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Advances. 2019;3(22):3780–3817. doi:10.1182/bloodadvances.2019000812.
- 3. Matzdorff A, Meyer O, Ostermann H, et al. Immune thrombocytopenia — current diagnostics and therapy: recommendations of a joint working group of DGHO, OGHO, SGH, GPOH, and DGTI. Annals of Hematology. 2018;97(9):1583–1604.
- 4. Miltiadous O, Hou M, Bussel JB. Identifying and treating refractory ITP: difficulty in diagnosis and role of combination treatment. Blood. 2020;135(7):472–490. doi:10.1182/blood.2019003599.
- 5. Royal College of Pathologists of Australasia (RCPA). Peripheral blood film — recommendations. RCPA Manual. 10th ed. Surry Hills, NSW: RCPA; 2023.
- 6. Australian Institute of Health and Welfare (AIHW). Aboriginal and Torres Strait Islander Health Performance Framework: Blood and blood-forming organ diseases. Cat. no. IHW 256. Canberra: AIHW; 2023.
- 7. 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(11):2386–2393. doi:10.1182/blood-2008-07-162503.
- 8. Australasian Society of Clinical Immunology and Allergy (ASCIA). Guidelines for vaccination of asplenic and hyposplenic individuals. Sydney: ASCIA; 2024.
- 9. Ghanima W, Godeau B, Bussel JB, et al. Immune thrombocytopenia: no longer a diagnosis of exclusion. Lancet Haematology. 2022;9(9):e671–e682. doi:10.1016/S2352-3026(22)00218-6.
- 10. Federal Register of Legislation. National Health (Pharmaceutical Benefits) Regulations 2023. Commonwealth of Australia, Canberra. Available at: https://www.legislation.gov.au.
- 11. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health, Canberra: 2024. Available at: https://immunisationhandbook.health.gov.au.
- 12. Samuelson Bannow BS, Lee A, Khorana AA, et al. Management of cancer-associated thrombocytopenia with thrombopoietin receptor agonists. American Society of Clinical Oncology Educational Book. 2023;43:e390112.
- 13. Royal Australian College of General Practitioners (RACGP). Red Book: Guidelines for preventive activities in general practice. 9th ed. East Melbourne: RACGP; 2018 (updated 2023).