What is the recommended treatment for immune thrombocytopenic purpura (ITP)?

Treatment of Immune Thrombocytopenic Purpura (ITP)

For patients with ITP, first-line treatment should include corticosteroids, with longer courses preferred over shorter courses, or IVIg when a more rapid increase in platelet count is required. 1

Indications for Treatment

• Treatment is generally indicated for patients with platelet counts <30 × 10⁹/L 1
• Treatment decisions should be based primarily on bleeding symptoms rather than platelet count alone 2
• Patients with active bleeding, regardless of platelet count, require immediate intervention

First-Line Treatment Options

Corticosteroids

• Preferred initial therapy for most patients with response rates of 60-80% initially 2
• Options include:
  • Prednisone 1-2 mg/kg/day for up to 14 days 2
  • High-dose dexamethasone (4 mg/kg/day for 3-4 days) 2
• Sustained responses only seen in 20-40% of patients 2
• Prolonged corticosteroid use should be avoided due to significant side effects 2

Intravenous Immunoglobulin (IVIg)

• Indicated when a more rapid increase in platelet count is required 1
• Initial dose: 1 g/kg as a one-time dose (may be repeated if necessary) 1
• Response rate >80% of patients, typically within 24-48 hours 1, 2
• Advantages:
  • Faster response than corticosteroids
  • Effective in patients who fail corticosteroid therapy
• Disadvantages:
  • Side effects include headaches, requiring prolonged infusion 1
  • Rare but serious toxicities include renal failure and thrombosis 1

Anti-D Immunoglobulin

• Can be used in Rh(D)-positive patients as an alternative to IVIg 1
• Administered as a short infusion 1
• Should not be used in patients who have undergone splenectomy 2

Emergency Treatment for Severe Bleeding

For patients with uncontrolled bleeding or at high risk of bleeding:

• Combination of prednisone and IVIg is recommended 1
• High-dose methylprednisolone may also be effective 1
• Other rapid options include:
  • Platelet transfusions (possibly combined with IVIg)
  • Emergency splenectomy
  • Vinca alkaloids (for rapid response) 1

Second-Line Treatment Options

For patients who fail first-line therapy or relapse:

Splenectomy

• Recommended for patients who have failed corticosteroid therapy 1
• Initial response rate of approximately 80% with about two-thirds maintaining long-term response 3
• Both laparoscopic and open splenectomy offer similar efficacy 1
• Complications include risk of infection and thrombosis

Thrombopoietin Receptor Agonists (TPO-RAs)

• Recommended for patients at risk of bleeding who:
  • Relapse after splenectomy OR
  • Have a contraindication to splenectomy AND
  • Have failed at least one other therapy 1
• May also be considered for patients who have failed first-line therapy without splenectomy 1
• Examples include romiplostim (Nplate) 3
• Caution: TPO-RAs may increase risk of blood clots if platelet count becomes too high 3
• Requires regular monitoring of platelet counts 2

Rituximab

• Consider for patients at risk of bleeding who have failed first-line therapy or splenectomy 1
• Response rate: 50-60% short-term, with 20-30% maintaining long-term response 2

Management of Secondary ITP

• H. pylori-associated ITP: Eradication therapy should be administered if H. pylori infection is confirmed 1
• HCV-associated ITP: Consider antiviral therapy; initial ITP treatment should be IVIg 1
• HIV-associated ITP: Treat HIV infection with antiretroviral therapy before other treatments unless significant bleeding is present 1

Special Populations

Pregnant Patients

• Recommended treatments: corticosteroids or IVIg 1
• Mode of delivery should be based on obstetric indications, not platelet count 1

Asymptomatic Post-Splenectomy Patients

• No further treatment needed if platelet counts >30 × 10⁹/L 1

Treatment Algorithm

1. Initial assessment: Determine if treatment is needed based on platelet count (<30 × 10⁹/L) and/or bleeding symptoms
2. First-line therapy:
   • For most patients: Corticosteroids (prednisone 1-2 mg/kg/day)
   • If rapid platelet increase needed: IVIg (1 g/kg)
3. If no response or relapse after first-line therapy:
   • Consider splenectomy (preferred second-line option)
   • Alternative second-line options: TPO-RAs or rituximab
4. For refractory ITP (failure of multiple therapies):
   • TPO-RAs if not tried previously
   • Immunosuppressants (azathioprine, cyclosporin A, mycophenolate mofetil)
   • Combination therapies

Monitoring

• Weekly platelet count monitoring during dose adjustment phase
• Monthly monitoring after establishing stable dose 2
• Follow-up with a hematologist within 24-72 hours of hospital discharge 2