What is the management of Immune Thrombocytopenic Purpura (ITP)?

Management of Immune Thrombocytopenic Purpura (ITP)

Corticosteroids are the first-line treatment for ITP, with IVIg recommended when rapid platelet count increase is needed, followed by splenectomy for those who fail initial therapy. 1

Initial Assessment and Diagnosis

• Complete blood count with peripheral blood smear review
• Coagulation studies (PT, PTT, fibrinogen)
• Testing for secondary causes:
  • HIV serology
  • Hepatitis B and C serology
  • H. pylori testing
  • Blood type and Rh(D) typing if anti-D immunoglobulin is considered 2

First-Line Treatment Options

Corticosteroids

• Prednisone 1-2 mg/kg/day for a maximum of 14 days 1
• Higher doses (4 mg/kg/day for 3-4 days) may be effective in up to 75% of patients 1
• Response typically occurs within 2-7 days 1
• Short course (≤6 weeks including taper) strongly recommended over prolonged courses 2
• Monitor for side effects: hyperglycemia, hypertension, mood changes, gastritis, weight gain 1, 2

Intravenous Immunoglobulin (IVIg)

• Recommended when rapid increase in platelet count is required 1
• Can be used in conjunction with corticosteroids for faster response 1
• Dose: 1 g/kg as a one-time dose, may be repeated if necessary 1
• Effective in >80% of patients with response in 1-2 days 1
• Side effects include headache, fever 1

Intravenous Anti-D Immunoglobulin

• Option for Rh(D)-positive patients only 1
• Can be given as a short infusion 1
• Side effects include mild extravascular hemolysis 1
• Should be used if corticosteroids are contraindicated 1

Second-Line Treatment Options

Splenectomy

• Recommended for patients who have failed corticosteroid therapy 1
• Both laparoscopic and open splenectomy offer similar efficacy 1
• Vaccination against encapsulated organisms required before procedure 1

Thrombopoietin Receptor Agonists

• Recommended for patients who:

  • Relapse after splenectomy
  • Have contraindications to splenectomy
  • Have failed at least one other therapy 1

• Romiplostim (Nplate):

  • Starting dose: 1 mcg/kg subcutaneously weekly 3
  • Adjust dose to maintain platelet count >50 × 10⁹/L 3
  • Durable response in 61% of non-splenectomized patients and 38% of splenectomized patients 2
  • Monitor for risk of blood clots if platelet count becomes high 3
  • Weekly monitoring during dose adjustments, monthly after stable dose 2

Rituximab

• May be considered for patients at risk of bleeding who have failed corticosteroids, IVIg, or splenectomy 1

Management of Secondary ITP

HCV-Associated ITP

• Consider antiviral therapy in the absence of contraindications 1
• If ITP treatment is required, initial treatment should be IVIg 1
• Monitor platelet count closely due to risk of worsening thrombocytopenia with interferon 1

HIV-Associated ITP

• Treat HIV infection with antiviral therapy before other treatment options 1
• If ITP treatment is required, use corticosteroids, IVIg, or anti-D 1

H. pylori–Associated ITP

• Screen for H. pylori in patients with ITP 1
• Administer eradication therapy in patients with confirmed H. pylori infection 1

ITP During Pregnancy

• Pregnant patients requiring treatment should receive either corticosteroids or IVIg 1
• Mode of delivery should be based on obstetric indications, not platelet count 1

Monitoring and Follow-up

• Target platelet count >30-50 × 10⁹/L to prevent bleeding 2
• Daily platelet counts until stable in hospitalized patients 2
• Weekly monitoring during dose adjustments in outpatients 2
• Follow-up with a hematologist within 24-72 hours of discharge 2

Treatment Algorithm

1. Start with prednisone 1-2 mg/kg/day for 2-4 weeks (maximum 14 days)
2. Add IVIg 1 g/kg if rapid increase in platelet count needed
3. If no response or relapse after initial therapy, consider:
   • Splenectomy (first choice for second-line)
   • Thrombopoietin receptor agonists if splenectomy contraindicated
   • Rituximab as alternative second-line option
4. For refractory cases, consider combination therapies or clinical trials

Common Pitfalls and Caveats

• Treating based on platelet count alone rather than bleeding risk
• Prolonged corticosteroid use leading to significant side effects
• Delaying splenectomy in appropriate candidates who have failed first-line therapy
• Not screening for secondary causes of ITP (HIV, HCV, H. pylori)
• Failure to monitor for complications of therapy, especially with thrombopoietin receptor agonists