Is splenectomy a treatment option for Immune Thrombocytopenic Purpura (ITP)?

Splenectomy for Immune Thrombocytopenia (ITP)

Yes, splenectomy is a recommended second-line treatment option for ITP, particularly for patients who have failed corticosteroid therapy, with a high initial response rate of 85%, though up to 30% of responders may relapse within 10 years. 1

Role of Splenectomy in ITP Treatment Algorithm

Splenectomy works by removing the primary site of platelet clearance and autoantibody production in ITP. The treatment approach follows this sequence:

1. First-line therapy: Corticosteroids and/or IVIG

   • Longer courses of corticosteroids are preferred as first-line treatment 1
   • Response rates: 60-70% for corticosteroids, 90% for IVIG 1
   • Responses are typically transient (<6 months for steroids, 2-4 weeks for IVIG) 1

2. Second-line therapy options when first-line fails:

   • Splenectomy (Grade 1B recommendation) 1
   • Thrombopoietin receptor agonists (TPO-RAs) 1
   • Rituximab (Grade 2C recommendation) 1

Efficacy and Considerations for Splenectomy

Benefits:

• Highest initial response rate among second-line therapies (85%) 1
• Highest rate of durable long-term remission (50-70%) 2
• Potential for medication-free remission 2
• Recommended by American Society of Hematology guidelines for patients who fail corticosteroid therapy 1

Risks and Limitations:

• Surgical complications in 10% of patients within 30 days post-procedure 1
• Long-term risks including:
  • 3-fold increased risk of septicemia
  • 4.5-fold increased risk of pulmonary embolism
  • 4.7-fold increased risk of non-Hodgkin lymphoma 1
  • 1.6-3.1-fold increased risk of sepsis 1
  • Need for lifelong management to prevent sepsis (vaccinations, prophylactic antibiotics) 1
• No reliable predictors of response 2

Timing of Splenectomy

• Ideally delayed for at least 12 months after diagnosis to allow for spontaneous or therapy-induced remissions 2
• Both laparoscopic and open splenectomy offer similar efficacy (Grade 1C recommendation) 1
• Laparoscopic approach is associated with shorter hospital stays (average 3.2 days) and acceptable complication rates (5%) 3

Alternative Second-Line Options

TPO-RAs (eltrombopag, romiplostim):

• Response rates: 70-80% of patients 1
• Well-tolerated even with long-term use 1
• Recommended for patients at risk of bleeding who:
  • Relapse after splenectomy, or
  • Have contraindications to splenectomy and have failed at least one other therapy (Grade 1B) 1
  • May be considered before splenectomy in patients who have failed first-line therapy (Grade 2C) 1

Rituximab:

• Complete response in 40% of patients at one year, 20% at 3-5 years 4
• May be considered for patients who have failed first-line therapy (Grade 2C) 1

Patient Selection for Splenectomy

Splenectomy may be particularly appropriate for:

• Patients desiring freedom from medication and monitoring 2
• Those with fulminant ITP not responding well to medical therapy 2
• Patients who have failed corticosteroids, IVIG, or anti-D 1

Post-Splenectomy Management

• No further treatment needed in asymptomatic patients with platelet counts >30 × 10⁹/L (Grade 1C) 1
• Monitor for relapse and infectious complications
• Positive predictors of long-term remission: immediate postoperative platelet count surge or platelet count ≥100,000/μL 3

Key Pitfalls to Avoid

1. Performing splenectomy too early (<12 months from diagnosis) when spontaneous remission might still occur
2. Failing to provide appropriate vaccinations before splenectomy
3. Not considering patient age and comorbidities (older patients have increased surgical risks and lower response rates) 2
4. Overlooking accessory spleens (present in approximately 5% of patients) 3
5. Neglecting long-term infection prevention strategies post-splenectomy

While splenectomy use has declined with the advent of medical alternatives like TPO-RAs and rituximab, it remains an important treatment option with the highest rate of durable response for appropriately selected patients with ITP.