What is the treatment for an Immune Thrombocytopenic Purpura (ITP) flare-up?

Treatment for ITP Flare-Up

For an ITP flare-up requiring treatment, initiate corticosteroids as first-line therapy, adding IVIG (1 g/kg) when rapid platelet increase is needed for bleeding risk or urgent procedures. 1

Initial Assessment: Determine If Treatment Is Needed

Not all ITP flare-ups require treatment—the decision to treat should be based on bleeding symptoms and bleeding risk, not platelet count alone. 2, 3

Treatment is indicated when: 1, 2

• Active significant bleeding is present
• Platelet count <30,000/mcL with high bleeding risk factors
• Platelet count <50,000/mcL with documented bleeding symptoms (mucous membrane bleeding)

Observation without treatment is appropriate for: 2, 3

• Asymptomatic patients without bleeding symptoms, regardless of platelet count
• This avoids the common pitfall of treating platelet numbers rather than clinical bleeding risk

First-Line Treatment Options

Corticosteroids (Preferred Initial Therapy)

Longer courses of corticosteroids are preferred over shorter courses or IVIG alone as first-line treatment. 1 The American Society of Hematology recommends this approach with Grade 2B evidence. 1

Dosing options include: 4

• Prednisone 0.5-2 mg/kg/day for 2-4 weeks
• Dexamethasone 40 mg/day for 4 days

IVIG: When to Add or Use Alone

IVIG should be used WITH corticosteroids when a more rapid increase in platelet count is required (Grade 2B), such as before urgent procedures or with active bleeding. 1

Standard IVIG dosing: 1 g/kg as a one-time dose, which may be repeated if necessary. 1

IVIG or anti-D can be used as first-line treatment ONLY if corticosteroids are contraindicated (Grade 2C). 1 This is not routine first-line therapy—IVIG is expensive and should be reserved for specific indications. 2

Research supports this approach: a randomized trial of 122 adults with severe AITP showed IVIG combined with oral prednisone resulted in platelet counts >50 × 10⁹/L for 18.5 days, demonstrating superior efficacy. 5

Anti-D Immunoglobulin

Anti-D can be used as an alternative to IVIG in appropriate patients (Rh-positive, non-splenectomized) when corticosteroids are contraindicated. 1

Second-Line Treatment for Refractory or Relapsed ITP

After Failed Corticosteroid Therapy

Splenectomy is recommended for patients who have failed corticosteroid therapy (Grade 1B), providing long-term responses in 60-70% of patients. 1, 4

Thrombopoietin Receptor Agonists (TPO-RAs)

TPO-RAs are recommended for patients at risk of bleeding who: 1

• Relapse after splenectomy or have contraindications to splenectomy AND have failed at least one other therapy (Grade 1B)
• May be considered for those who failed one line of therapy (corticosteroids or IVIG) without splenectomy (Grade 2C)

Romiplostim (Nplate) is given subcutaneously once weekly with close monitoring of platelet counts. 6 Critical warning: TPO-RAs may cause dangerously high platelet counts leading to thrombotic complications including pulmonary embolism, myocardial infarction, and stroke. 6

Rituximab

Rituximab may be considered for patients at risk of bleeding who have failed one line of therapy (Grade 2C). 1

Special Populations

Pregnancy

Pregnant patients requiring treatment should receive either corticosteroids or IVIG (Grade 1C). 1, 4 Mode of delivery should be based on obstetric indications, not platelet count (Grade 2C). 1

Secondary ITP

Always screen for secondary causes before treating as primary ITP: 4

• HIV-associated: Treat HIV infection with antivirals first unless clinically significant bleeding is present (Grade 1A). 1
• HCV-associated: Consider antiviral therapy; if ITP treatment needed, use IVIG as initial treatment (Grade 2C). 1
• H. pylori-associated: Eradication therapy is recommended for positive testing (Grade 1B). 1, 4

Critical Pitfalls to Avoid

• Do not treat platelet numbers alone—treat bleeding risk and symptoms. 2
• Do not use IVIG as routine first-line monotherapy—it is expensive and should be reserved for rapid platelet increase needs or corticosteroid contraindications. 2
• Do not delay treatment in patients with active bleeding or high bleeding risk while waiting for platelet counts to trend. 2
• Monitor for thrombotic complications when using TPO-RAs, especially if platelet counts rise excessively. 6

Monitoring During Treatment

Close monitoring is essential: 6

• Check platelet counts weekly during dose adjustments
• Monthly monitoring once stable dosing achieved
• After stopping treatment, monitor for at least 2 weeks to detect rebound thrombocytopenia