What is the recommended treatment for Immune Thrombocytopenia (ITP)?

Treatment of Immune Thrombocytopenia (ITP)

For adult patients with ITP, a short course of corticosteroids (≤6 weeks) is recommended as first-line therapy, followed by thrombopoietin receptor agonists (TPO-RAs) such as eltrombopag or romiplostim as preferred second-line agents for those who are corticosteroid-dependent or unresponsive to corticosteroids. 1, 2

Diagnosis and Initial Assessment

• Diagnosis is one of exclusion after ruling out other causes of thrombocytopenia
• Initial workup should include:
  • Complete blood count with peripheral blood smear
  • Coagulation profile (PT, PTT, fibrinogen)
  • Liver and renal function tests
  • HIV and hepatitis B/C serology
  • H. pylori testing 2

Treatment Algorithm for Adult ITP

First-Line Treatment

• Corticosteroids: Short course (≤6 weeks including treatment and taper) 1

  • Preferred option: Dexamethasone 40 mg/day for 4 days (response rate 60-80%) 2
  • Alternative: Prednisone 0.5-2 mg/kg/day until platelet count increases to 30-50 × 10⁹/L 2
  • Caution: Avoid prolonged corticosteroid use due to significant adverse effects 1, 2

• For rapid platelet increase (active bleeding or procedures):

  • IVIG or IV anti-D can be combined with corticosteroids
  • Response typically occurs within 24-48 hours 2

Second-Line Treatment

For patients with ITP lasting ≥3 months who are corticosteroid-dependent or unresponsive:

1. TPO-RAs (preferred second-line option):

   • Either eltrombopag or romiplostim is recommended 1
   • Eltrombopag: Initial dose 36 mg orally once daily (18 mg for East/Southeast Asian patients or those with hepatic impairment) 3
   • Romiplostim: Initial dose 1 mcg/kg subcutaneously weekly, adjusted to maintain platelet count ≥50 × 10⁹/L (maximum 10 mcg/kg) 4
   • TPO-RAs are preferred over rituximab 1

2. Rituximab:

   • Preferred over splenectomy 1
   • But TPO-RAs are preferred over rituximab 1

3. Splenectomy:

   • Should be delayed for at least 1 year after diagnosis due to potential for spontaneous remission 1
   • Requires appropriate immunizations prior to procedure and antibiotic prophylaxis counseling 1

Special Considerations

Treatment Based on Platelet Count and Bleeding Risk

• Platelet count <20-30 × 10⁹/L: Treatment generally indicated due to significant bleeding risk 5
• Platelet count 30-50 × 10⁹/L with mucous membrane bleeding: Treatment indicated 5
• Higher platelet counts without bleeding: Observation may be appropriate 6

Viral-Associated Thrombocytopenia

• For HIV-associated thrombocytopenia: Antiretroviral therapy (HAART) is first-line 2
• For HCV-associated thrombocytopenia: Antiviral therapy is first-line 2

Follow-up and Monitoring

• Weekly platelet count monitoring during treatment initiation
• Monthly monitoring after establishing stable platelet counts
• Follow-up with a hematologist within 24-72 hours of diagnosis or disease relapse 1, 2

Treatment Pitfalls to Avoid

• Do not use TPO-RAs to normalize platelet counts - aim for ≥50 × 10⁹/L to reduce bleeding risk 4, 3
• Do not continue TPO-RAs if no response after 4 weeks at maximum dose 4
• Do not use prolonged corticosteroid courses (>6 weeks) due to significant adverse effects 1
• Do not delay hematology follow-up beyond 24-72 hours after diagnosis or relapse 1
• Do not use TPO-RAs for thrombocytopenia due to myelodysplastic syndrome 4, 3

The treatment approach should focus on reducing bleeding risk rather than normalizing platelet counts, with careful consideration of medication side effects and patient-specific factors such as comorbidities and medication preferences (oral vs. injectable).