Initial Treatment for Idiopathic Thrombocytopenic Purpura (ITP)
Corticosteroids are the standard first-line treatment for adults with newly diagnosed ITP requiring therapy, with prednisone (0.5-2 mg/kg/day) or high-dose dexamethasone (40 mg/day for 4 days) as the preferred initial agents. 1, 2, 3
When to Initiate Treatment
- Treatment is indicated when platelet count is <30 × 10⁹/L with bleeding symptoms, or <20 × 10⁹/L regardless of bleeding. 2, 3
- Patients older than 60 years and those with previous hemorrhage have higher bleeding risk and warrant earlier intervention. 2
- Treatment is rarely needed if platelet count >50 × 10⁹/L unless active bleeding is present, surgery is required, comorbidities predispose to bleeding, or anticoagulation is needed. 3
- Immediate treatment is required for patients with active CNS, GI, or genitourinary bleeding, or those requiring urgent surgery. 3
First-Line Corticosteroid Options
Prednisone
- Dose: 0.5-2 mg/kg/day until platelet count reaches 30-50 × 10⁹/L, then rapidly taper. 2, 3
- Expected response: 70-80% initial response rate, but only 20-40% sustained long-term response. 3, 4
- Corticosteroids should not be continued beyond 6-8 weeks for initial treatment. 2
High-Dose Dexamethasone
- Dose: 40 mg/day for 4 days, repeated every 14-28 days for up to 4-6 cycles. 2, 5, 6
- Dexamethasone achieves faster platelet response and potentially better tolerability than prednisone, with initial response rates up to 90% and sustained response of 50-80%. 3, 5
- Dexamethasone shows increased platelet count response at 7 days and higher remission rates compared to prednisone (relative risk 1.31 for platelet response and 2.96 for remission rates). 2
- When used as first-line therapy, 59% of patients remain in remission after 31 months. 5
Adjunctive First-Line Therapies
Intravenous Immunoglobulin (IVIG)
- IVIG should be used with corticosteroids when a more rapid increase in platelet count is required. 1, 2
- Dose: 1 g/kg as a single dose; may be repeated if necessary. 1, 2
- IVIG achieves platelet increase within 24 hours and can be combined with corticosteroids for enhanced response and reduced infusion reactions. 3
- IVIG or anti-D should be used as first-line treatment if corticosteroids are contraindicated. 1
Anti-D Immunoglobulin
- Only for Rh(D)-positive, non-splenectomized patients. 1, 3, 4
- Dose: 75 mcg/kg, providing predictable, transient platelet increases. 2, 3
Emergency Treatment Protocol
- For severe bleeding or platelet count <10 × 10⁹/L with high bleeding risk, combine prednisone and IVIG, with consideration of high-dose methylprednisolone for rapid response. 2
Special Populations
Pregnancy
- Pregnant patients requiring treatment should receive either corticosteroids or IVIG only (grade 1C recommendation). 1, 3
- Mode of delivery should be based on obstetric indications, not maternal platelet count. 1, 3
HIV-Associated ITP
- Treat underlying HIV infection with antiviral therapy before ITP-specific therapy unless clinically significant bleeding is present (grade 1A recommendation). 1, 3
- If ITP treatment is required, use corticosteroids, IVIG, or anti-D. 1
HCV-Associated ITP
- Consider antiviral therapy in the absence of contraindications, but closely monitor platelet count due to risk of worsening thrombocytopenia from interferon. 1
- If ITP treatment is required, initial treatment should be IVIG. 1
H. pylori-Associated ITP
- Eradication therapy should be administered for patients found to have H. pylori infection (grade 1B recommendation). 1
- Screening for H. pylori should be considered in ITP patients where eradication therapy would be used if positive. 1
Critical Corticosteroid Side Effects to Monitor
Short-term (weeks)
- Mood swings, weight gain, fluid retention, Cushingoid features, and hyperglycemia. 3
Long-term (months)
- Osteoporosis, avascular necrosis, hypertension, diabetes, skin changes, cataracts, and immunosuppression with opportunistic infections. 3
When First-Line Fails
- Patients are considered corticosteroid failures if they have no response after 4 weeks of treatment, platelet count drops below safe levels during taper, or require continuous corticosteroids to maintain platelet count. 2
- Patients requiring on-demand corticosteroids after completing induction should be considered non-responders and switched to second-line therapy. 2
- Splenectomy remains highly effective with 80% initial response and 60-65% long-term response. 3, 7
- Thrombopoietin receptor agonists (TPO-RAs) are increasingly preferred before splenectomy due to high response rates and potential for remission. 3, 7