What is the initial treatment for Immune Thrombocytopenic Purpura (ITP)?

Initial Treatment for Immune Thrombocytopenic Purpura (ITP)

Corticosteroids are the recommended first-line treatment for newly diagnosed adult ITP, with longer courses of prednisone preferred over shorter courses of corticosteroids or IVIG. 1, 2

First-Line Treatment Options for Adults

• Prednisone (1 mg/kg orally for 21 days followed by tapering) is the primary first-line therapy for newly diagnosed adult ITP 3
• Treatment decisions should be based on bleeding severity, bleeding risk, patient activity level, and patient preferences rather than platelet count alone 3
• Treatment is generally indicated for patients with platelet counts <30 × 10^9/L or those with significant bleeding symptoms 1, 2
• Initial response to corticosteroids occurs in 70-80% of patients, but sustained responses are seen in only 20-40% of cases 3, 4

Alternative First-Line Options

• Dexamethasone (40 mg/day for 4 days) can be considered as an alternative to prednisone when a more rapid platelet increase is needed 5

  • Dexamethasone works faster in increasing platelet counts and may have fewer adverse events due to shorter treatment duration 5
  • However, its superiority in achieving long-term remission compared to prednisone is not well established 5

• Intravenous immunoglobulin (IVIG) should be added to corticosteroids when a more rapid increase in platelet count is required 1, 2

  • The recommended initial dose is 1 g/kg as a one-time dose, which may be repeated if necessary 1
  • IVIG or anti-D immunoglobulin can be used as first-line treatment if corticosteroids are contraindicated 1

First-Line Treatment Options for Children

• For children with newly diagnosed ITP who have no or minor bleeding, observation is often preferred over immediate treatment 1
• When treatment is necessary for children with non-life-threatening mucosal bleeding or diminished quality of life, options include:
  • Corticosteroids (prednisone 2-4 mg/kg/d for 5-7 days) 1
  • IVIG (0.8-1 g/kg) 1, 2
  • Anti-D immunoglobulin (for Rh-positive, non-splenectomized patients) 1, 3

Special Considerations

• Before initiating treatment, testing for HCV and HIV is recommended for all patients with suspected ITP 1, 2
• Bone marrow examination is generally not necessary for patients presenting with typical ITP 1, 2
• Secondary causes of ITP should be ruled out, including antiphospholipid syndrome, autoimmune disorders, drug-induced thrombocytopenia, and infections 2
• For pregnant patients requiring treatment, either corticosteroids or IVIG is recommended 1

Common Pitfalls and Caveats

• Prolonged corticosteroid use (>6-8 weeks) should be avoided due to significant side effects including weight gain, mood alterations, hypertension, diabetes, osteoporosis, and increased infection risk 3
• The goal of treatment is to maintain a hemostatic platelet count (typically >30-50 × 10^9/L) rather than normalizing platelet counts 3
• Treatment decisions should be based primarily on bleeding symptoms rather than platelet count alone, though counts <20-30 × 10^9/L generally warrant treatment 3, 6
• The frequency of death from complications of treatment is similar to the frequency of death from bleeding, emphasizing the importance of judicious use of therapy 7

If patients fail to respond to initial corticosteroid therapy, second-line options include splenectomy (which has historically been the gold standard second-line therapy with long-term responses in approximately 60-70% of patients) or thrombopoietin receptor agonists (TPO-RAs) 1, 2, 3.