What is the recommended management for Immune Thrombocytopenic Purpura (ITP)?

Management of Immune Thrombocytopenic Purpura (ITP)

Corticosteroids are the standard first-line therapy for ITP, with prednisone at 0.5-2 mg/kg/day until platelet count increases (30-50 × 10^9/L), followed by rapid tapering to avoid complications. 1

First-Line Treatment Options

Adults with Newly Diagnosed ITP:

• Longer courses of corticosteroids are preferred over shorter courses as first-line treatment 1
• Prednisone is the standard initial therapy at 0.5-2 mg/kg/day until platelet count increases, which may take several days to weeks 1
• Dexamethasone (40 mg/day for 4 days) shows high initial response rates up to 90% with sustained responses in 50-80% of patients when given in 1-4 cycles 1, 2
• High-dose methylprednisolone has shown response rates as high as 95% with faster response times compared to prednisone 1
• Treatment should be initiated for patients with platelet counts <30 × 10^9/L 1
• Corticosteroids should be rapidly tapered and stopped in responders, and especially in non-responders after 4 weeks to avoid complications 1

When Rapid Platelet Increase is Required:

• IVIg should be used with corticosteroids when a more rapid increase in platelet count is required 1
• IVIg dose should initially be 1 g/kg as a one-time dose, which may be repeated if necessary 1
• IV anti-D (50-75 μg/kg) is appropriate for Rh(D) positive, non-splenectomized patients as an alternative to IVIg 1
• Either IVIg or anti-D should be used as first-line treatment if corticosteroids are contraindicated 1

Second-Line Treatment Options for Adults

For Patients Who Fail Initial Corticosteroid Therapy:

• Splenectomy is recommended for patients who have failed corticosteroid therapy 1
• Both laparoscopic and open splenectomy offer similar efficacy 1
• Proper immunizations prior to splenectomy and antibiotic prophylaxis after splenectomy are essential 1

Alternative Second-Line Options:

• Thrombopoietin receptor agonists (TPO-RAs) like romiplostim are recommended for patients at risk of bleeding who relapse after splenectomy or have contraindications to splenectomy 1
• TPO-RAs may be considered for patients who have failed one line of therapy such as corticosteroids or IVIg and have not had splenectomy 1
• Rituximab may be considered for patients at risk of bleeding who have failed one line of therapy 1
• Other immunosuppressive options include azathioprine (1-2 mg/kg), cyclosporin A, cyclophosphamide, danazol, dapsone, and mycophenolate mofetil 1

Emergency Treatment for Severe Bleeding

• Combined prednisone and IVIg are recommended for emergency treatment of patients with uncontrolled bleeding 1
• High-dose methylprednisolone may also be useful in emergency settings 1
• Other rapid-acting therapies include platelet transfusion (possibly combined with IVIg) and emergency splenectomy 1

Special Populations

Pregnancy:

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

Secondary ITP:

• For HCV-associated ITP: Consider antiviral therapy in the absence of contraindications; initial ITP treatment should be IVIg 1
• For HIV-associated ITP: Treatment of HIV infection with antiviral therapy should be considered before other treatment options; if ITP treatment is required, use corticosteroids, IVIg, or anti-D 1
• For H. pylori-associated ITP: Eradication therapy should be administered if H. pylori infection is detected 1
• Consider screening for H. pylori in ITP patients 1

Treatment After Splenectomy

• No further treatment is recommended in asymptomatic patients after splenectomy who have platelet counts >30 × 10^9/L 1
• For patients who relapse after splenectomy, TPO-RAs are recommended 1, 3

Monitoring and Follow-up

• During TPO-RA treatment, regular monitoring of platelet counts is essential to avoid thrombotic complications from excessive platelet increases 3
• After discontinuing TPO-RAs, blood tests should be performed for at least 2 weeks to check for platelet count drops 3
• Patients should be monitored for bleeding during all phases of treatment 3

Common Pitfalls and Caveats

• Long-term corticosteroid use leads to significant adverse effects including mood swings, weight gain, diabetes, hypertension, osteoporosis, and immunosuppression 1, 2
• Splenectomy, while effective, carries surgical risks and may predispose patients to sepsis and rare but potentially fatal infections 4, 5
• TPO-RAs may increase risk of blood clots if platelet count becomes too high during treatment 3
• IVIg can cause rare but serious toxicities including renal failure and thrombosis 1
• The goal of treatment is not to normalize platelet count but to maintain a safe level to prevent bleeding (typically >30 × 10^9/L) 4, 6