Immune Thrombocytopenia (ITP): Symptoms, Severity Grading, and Management
Immune Thrombocytopenia (ITP) is an autoimmune disorder with antibody-mediated platelet destruction and impaired production, leading to isolated thrombocytopenia (<100×10⁹/L) without other cytopenias.
Clinical Presentation
- Mucocutaneous bleeding: Petechiae (pinpoint, non-blanching), purpura, ecchymoses
- Epistaxis: Often prolonged, recurrent
- Gingival bleeding
- Menorrhagia
- Hematuria, GI bleeding (less common)
- Fatigue: Common, multifactorial (cytokines, anemia from bleeding, disease burden)
- Asymptomatic: Many diagnosed incidentally on CBC
Severity Classification (International Consensus)
| Grade | Platelet Count | Bleeding | Management |
|---|
| Mild | 30–100×10⁹/L | Minimal/none | Observation |
| Moderate | 10–30×10⁹/L | Mucocutaneous | Consider treatment |
| Severe | <10×10⁹/L | Significant/spontaneous | Treat |
| Life-threatening | Any | ICH, visceral bleed | Emergent treatment |
Red Flags Requiring Urgent Care
- Intracranial hemorrhage (headache, vomiting, altered consciousness, focal deficits)
- Severe GI/GU bleeding
- Platelets <10×10⁹/L with wet bleeding
- Wet purpura (blood blisters in mouth)
Diagnostic Approach
- Exclusion diagnosis: No single confirmatory test
- CBC + peripheral smear (exclude pseudothrombocytopenia, schistocytes, blasts)
- Rule out: Drug-induced (heparin, quinine, sulfonamides), HIV, HCV, H. pylori, SLE, ALPS, CLL, MDS, TTP/HUS (ADAMTS13), pregnancy (HELLP)
- Bone marrow biopsy: Not routine; if atypical features, >60 years, treatment failure
Treatment (ASH 2019 / IWG Guidelines)
First-Line
- Corticosteroids: Prednisone 1 mg/kg/day × 2–4 weeks → taper; or dexamethasone 40 mg/day × 4 days × 1–4 cycles
- IVIG: 1 g/kg/day × 1–2 days (rapid rise; transient 2–4 weeks); pre-procedure, pregnancy, contraindication to steroids
- Anti-D immunoglobulin: 50–75 µg/kg (Rh+ non-splenectomized); hemolysis risk
Second-Line (Relapsed/Refractory)
- TPO-receptor agonists (TPO-RAs): Romiplostim (SC weekly), Eltrombopag (daily oral), Avatrombopag (daily oral) → sustained response, long-term use
- Rituximab: 375 mg/m² weekly × 4; ~60% response, ~20–30% long-term remission; infection risk
- Splenectomy: ~60–70% durable remission; lifelong infection risk (encapsulated organisms); vaccinate pre-op
- Fostamatinib: Syk inhibitor; oral; ~20% response
Emergent (Life-Threatening Bleeding)
- IVIG 1 g/kg + high-dose steroids + platelet transfusion (large volume, HLA-matched if possible) ± TPO-RA ± rFVIIa
Special Populations
- Pregnancy: Gestational thrombocytopenia (mild, >70×10⁹/L) vs. ITP; avoid TPO-RAs, rituximab; IVIG, steroids preferred
- Children: Often acute post-viral, self-limiting (80% remit in 6 months); observe if mild
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