What is the treatment for thrombocytopenia (low platelet count)?

Treatment for Thrombocytopenia (Low Platelet Count)

The treatment for thrombocytopenia depends on the cause, severity of symptoms, and platelet count, with first-line therapy for immune thrombocytopenia (ITP) consisting of corticosteroids or intravenous immunoglobulin (IVIg) when treatment is required. 1

Diagnostic Approach

Before initiating treatment, it's crucial to determine the underlying cause:

• Primary (Immune) Thrombocytopenia: Diagnosis of exclusion with platelet count <100 × 10⁹/L
• Secondary Thrombocytopenia: Associated with autoimmune diseases, viral infections (HCV, HIV), medications, liver disease, or pregnancy 1, 2

Key diagnostic steps:

• Rule out pseudothrombocytopenia by collecting blood in heparin or sodium citrate tube
• Distinguish acute from chronic thrombocytopenia by reviewing previous platelet counts
• Test for HCV and HIV as recommended by guidelines 1
• Bone marrow examination is generally not necessary in typical ITP presentation 1

Treatment Algorithm Based on Cause and Severity

1. Immune Thrombocytopenia (ITP)

For patients with no bleeding or mild bleeding:

• Observation alone regardless of platelet count 1

For patients requiring treatment (significant bleeding or very low counts):

First-line therapy:

• Corticosteroids (longer courses preferred) or
• IVIg (0.8-1 g/kg as one-time dose) if rapid increase in platelet count is needed 1, 3
• Anti-D immunoglobulin for Rh-positive, non-splenectomized patients (not if hemoglobin is decreased due to bleeding) 1

Second-line therapy (if inadequate response to first-line):

• Rituximab (375 mg/m² weekly for 4 weeks) 3
• High-dose dexamethasone 1
• Thrombopoietin receptor agonists like romiplostim (Nplate) for patients who have failed corticosteroids, immunoglobulins, or splenectomy 4
• Initial dose: 1 mcg/kg subcutaneously weekly
• Adjust dose to maintain platelet count ≥50 × 10⁹/L
• Maximum dose: 10 mcg/kg weekly 4

Third-line therapy:

• Splenectomy for chronic or persistent ITP with significant bleeding and lack of response to other therapies 1
• Delay splenectomy for at least 12 months unless severe disease is present 1

2. Secondary Thrombocytopenia

• Lupus-associated thrombocytopenia: Treat with glucocorticoids and immunosuppressive agents (AZA, MMF, or cyclosporine) 1
• Drug-induced thrombocytopenia: Discontinue the offending medication 2
• Liver disease-related thrombocytopenia: Manage underlying liver condition 1, 2

3. Platelet Transfusion Guidelines

Platelet transfusion is indicated in:

• Active hemorrhage
• Platelet count <10 × 10⁹/L 2
• Before procedures:
  • Central venous catheter placement: if platelets <20 × 10⁹/L 1
  • Lumbar puncture: if platelets <50 × 10⁹/L 1

Monitoring and Follow-up

• Weekly complete blood counts (CBCs) during dose adjustment phase
• Monthly CBCs after establishing stable dose
• Continue monitoring for at least 2 weeks after discontinuing treatment 1, 4

Important Considerations and Pitfalls

1. Avoid excessive platelet counts: Thrombopoietin receptor agonists can cause thrombotic complications if platelet counts exceed 400 × 10⁹/L 4

2. Recognize thrombosis with thrombocytopenia syndromes: Some conditions (antiphospholipid syndrome, heparin-induced thrombocytopenia) can present with both thrombocytopenia and thrombosis 5

3. Activity restrictions: Patients with platelet counts <50 × 10⁹/L should avoid activities with high risk of trauma 2

4. Prophylactic platelet transfusions: Evidence for prophylactic transfusions before procedures is limited, and transfusions carry risks including transfusion-related lung injury 1

5. Pregnancy considerations: For pregnant women with ITP, treatment with corticosteroids or IVIg is recommended, and delivery method should be based on obstetric indications 1

By following this structured approach to diagnosis and treatment, most patients with thrombocytopenia can be effectively managed with improved outcomes in terms of morbidity, mortality, and quality of life.