Initial Treatment for Idiopathic Thrombocytopenic Purpura (ITP)
Corticosteroids are the standard first-line treatment for newly diagnosed ITP in adults, with prednisone 0.5-2 mg/kg/day or high-dose dexamethasone 40 mg/day for 4 days as the preferred initial options. 1, 2
When to Initiate Treatment
Treatment decisions should be based on bleeding symptoms and platelet count thresholds, not platelet count alone:
- Treat when platelet count is <20-30 × 10⁹/L with bleeding symptoms (mucosal bleeding, petechiae, purpura) 1, 2
- Treat when platelet count is <10-20 × 10⁹/L regardless of bleeding symptoms 1
- Patients >60 years or with previous hemorrhage have higher bleeding risk and warrant earlier intervention 2
- Immediate treatment is required for active CNS, GI, or genitourinary bleeding 3
Patients with platelet counts >30 × 10⁹/L who are asymptomatic or have only minor purpura do not routinely require treatment, as spontaneous remissions can occur 1
First-Line Corticosteroid Regimens
Standard Prednisone
- Dose: 0.5-2 mg/kg/day orally until platelet count reaches 30-50 × 10⁹/L 1, 2
- Rapidly taper after response; discontinue in non-responders after 4 weeks 1
- Expected outcomes: 70-80% initial response rate, but only 20-40% sustained long-term response 2, 3
- Duration: Should not exceed 6-8 weeks for initial treatment 2
High-Dose Dexamethasone (Preferred for Faster Response)
- Dose: 40 mg/day orally for 4 days, may repeat every 2-4 weeks for 1-4 cycles 1, 2
- Expected outcomes: Up to 90% initial response rate, 50-80% sustained response with 3-6 cycles 1, 2, 3
- Advantages: Faster platelet response (several days vs. several weeks), potentially better tolerability with short-term bolus therapy 1, 2
The American Society of Hematology recommends longer courses of corticosteroids over shorter courses because they are associated with longer time to loss of response 1
Adjunctive First-Line Therapies
Intravenous Immunoglobulin (IVIG)
- Use IVIG with corticosteroids when more rapid platelet increase is required (within 24 hours) 1, 2
- Dose: 1 g/kg as a single dose; may repeat if necessary 1, 2, 3
- Use IVIG alone as first-line treatment if corticosteroids are contraindicated 1
- Combining IVIG with corticosteroids enhances response and reduces infusion reactions 3
Anti-D Immunoglobulin
- Only appropriate for Rh(D)-positive, non-splenectomized patients 1
- Dose: 75 mcg/kg 3
- Provides predictable, transient platelet increases 3, 4
- Avoid in patients with autoimmune hemolytic anemia to prevent exacerbation of hemolysis 1
- Requires blood group, direct antiglobulin test (DAT), and reticulocyte count before administration 1
Emergency Treatment Protocol
For severe bleeding or platelet count <10 × 10⁹/L with high bleeding risk:
- Combine high-dose corticosteroids (prednisone or methylprednisolone) with IVIG 2
- Consider high-dose methylprednisolone 30 mg/kg/day for rapid response 1
- Add platelet transfusions for life-threatening bleeding 1
- Provide conventional critical care measures 1
Special Population Considerations
Pregnancy
- Use corticosteroids or IVIG only as first-line treatment (Grade 1C recommendation) 1, 2, 3
- Mode of delivery should be based on obstetric indications, not maternal platelet count 1, 2, 3
HIV-Associated ITP
- Treat underlying HIV infection with antivirals first before ITP-specific therapy unless clinically significant bleeding is present (Grade 1A recommendation) 1, 2, 3
- If ITP treatment is required, use corticosteroids, IVIG, or anti-D 1
HCV-Associated ITP
- Consider antiviral therapy in the absence of contraindications 1
- Monitor platelet count closely due to risk of worsening thrombocytopenia from interferon 1
- If ITP treatment is required, use IVIG as initial treatment 1, 3
H. pylori-Associated ITP
- Screen for H. pylori in patients with ITP 1
- Administer eradication therapy for patients with confirmed H. pylori infection (Grade 1B recommendation) 1
Critical Pitfalls to Avoid
- Do not continue corticosteroids beyond 6-8 weeks for initial treatment 2
- Patients requiring on-demand corticosteroids after completing induction should be considered non-responders and switched to second-line therapy 2
- Do not perform bone marrow examination routinely before initiating treatment; it is unnecessary in patients with typical ITP features 1
- Bone marrow examination is only indicated if there are atypical findings or abnormalities beyond isolated thrombocytopenia 1
Corticosteroid Side Effects to Monitor
Short-term toxicities include mood swings, weight gain, anger, anxiety, insomnia, Cushingoid facies, dorsal fat pad, diabetes, fluid retention, hypertension, and GI distress 1, 2, 3
Long-term toxicities include osteoporosis, skin thinning, alopecia, avascular necrosis, immunosuppression with opportunistic infections, psychosis, cataracts, and adrenal insufficiency 1, 2, 3
Defining Treatment Failure
Patients are considered corticosteroid failures if:
- No response after 4 weeks of treatment 2
- Platelet count drops below safe levels during taper 2
- Require continuous corticosteroids to maintain platelet count 2
Required Diagnostic Testing
Before initiating treatment:
- Test all patients for HCV and HIV (Grade 1B recommendation) 1
- Perform complete blood count and peripheral blood smear examination 1
- Obtain abdominal CT or ultrasound if splenomegaly is suspected on physical examination 1
- Blood group, DAT, and reticulocyte count are required before treating with anti-D 1