What is the initial workup and treatment for a patient with Immune Thrombocytopenic Purpura (ITP)?

Initial Workup and Treatment for Immune Thrombocytopenic Purpura (ITP)

The initial workup for ITP should include a complete blood count, peripheral blood smear examination, testing for HCV and HIV, while first-line treatment consists of corticosteroids for most patients, with IVIG added when rapid platelet increase is needed. 1

Diagnostic Workup

• The diagnosis of ITP is primarily based on history, physical examination, complete blood count, and examination of the peripheral blood smear to exclude other causes of thrombocytopenia 1
• Testing for HCV and HIV is strongly recommended for all patients with suspected ITP (grade 1B) 1
• The peripheral blood smear should show normal platelet morphology with possibly some large platelets, and no increased schistocytes 2
• A bone marrow examination is not necessary irrespective of age for patients presenting with typical ITP (grade 2C) 1
• Further investigations are only suggested if there are abnormalities other than thrombocytopenia (and perhaps findings of iron deficiency) in the blood count or smear 1
• For patients with risk factors, liver function tests are appropriate, especially to rule out conditions like preeclampsia in pregnant women 1
• Screening for H. pylori should be considered in patients with ITP in whom eradication therapy would be used if testing is positive (grade 2C) 1

First-Line Treatment

General Principles

• Treatment is not required for asymptomatic patients with platelet counts >30,000/μL who have only minor purpura 1, 3
• Treatment is indicated for patients with:
  • Platelet counts <10,000/μL 1
  • Platelet counts <20,000/μL with significant mucous membrane bleeding 1
  • Active bleeding regardless of platelet count 3
  • Need for invasive procedures 3

First-Line Treatment Options

• Corticosteroids are the standard initial therapy for most adult patients with ITP 3
• Intravenous Immunoglobulin (IVIG) should be:
  • Used with corticosteroids when a more rapid increase in platelet count is required (grade 2B) 1
  • Used as first-line treatment if corticosteroids are contraindicated (grade 2C) 1
  • Administered at an initial dose of 1 g/kg as a one-time dose; this dosage may be repeated if necessary (grade 2B) 1
• Anti-D immunoglobulin can be used as a first-line treatment in appropriate patients (Rh-positive, non-splenectomized) if corticosteroids are contraindicated (grade 2C) 1

Management of Severe or Life-Threatening Bleeding

• Patients with severe, life-threatening bleeding should be hospitalized and receive:
  • High-dose parenteral glucocorticoid therapy
  • IVIG
  • Platelet transfusions 1
• Hospitalization is appropriate for patients with platelet counts <20,000/μL who have significant mucous membrane bleeding 1

Secondary ITP Management

• HCV-associated ITP:
  • Antiviral therapy should be considered in the absence of contraindications (grade 2C)
  • If ITP treatment is required, the initial treatment should be IVIG (grade 2C) 1
• HIV-associated ITP:
  • Treatment of HIV infection with antiviral therapy should be considered before other treatment options unless the patient has clinically significant bleeding complications (grade 1A)
  • If ITP treatment is required, initial treatment should consist of corticosteroids, IVIG, or anti-D (grade 2C) 1
• H. pylori–associated ITP:
  • Eradication therapy should be administered for patients who are found to have H. pylori infection (grade 1B) 1

Second-Line Treatment Options

• For patients who fail initial corticosteroid therapy, options include:
  • Splenectomy (grade 1B) 1
  • Thrombopoietin receptor agonists for patients at risk of bleeding who have failed one line of therapy such as corticosteroids or IVIG (grade 2C) 1
  • Rituximab may be considered for patients at risk of bleeding who have failed one line of therapy (grade 2C) 1

Special Considerations

Pregnancy

• Pregnant women with ITP and platelet counts >50,000/μL do not routinely require treatment 1
• Pregnant patients requiring treatment should receive either corticosteroids or IVIG (grade 1C) 1
• For pregnant women with ITP, the mode of delivery should be based on obstetric indications (grade 2C) 1

Common Pitfalls and Caveats

• Misdiagnosis of myelodysplastic syndrome with isolated thrombocytopenia (MDS-IT) as ITP is relatively common in refractory cases 4
• Patients with persistent thrombocytopenia unresponsive to first-line therapy should undergo more thorough evaluation, including bone marrow examination and cytogenetic testing 4
• Romiplostim (thrombopoietin receptor agonist) is not indicated for the treatment of thrombocytopenia due to myelodysplastic syndrome or any cause of thrombocytopenia other than ITP 5
• Patients treated with thrombopoietin receptor agonists should be monitored for thrombotic/thromboembolic complications, particularly those with chronic liver disease who may develop portal vein thrombosis 5