What is the management plan for a patient with Immune Thrombocytopenic Purpura (ITP) and severe thrombocytopenia, presenting with hematuria?

Emergency Management of ITP with Severe Thrombocytopenia (Platelet Count 3 × 10⁹/L) and Hematuria

This patient requires immediate hospital admission and urgent treatment with combined corticosteroids and IVIG to rapidly increase the platelet count and control bleeding. 1

Immediate Actions

Hospital Admission

• Admit this patient immediately - with a platelet count <20 × 10⁹/L and active bleeding (hematuria), hospital admission is strongly indicated regardless of whether this is newly diagnosed or established ITP 1
• Patients with platelet counts this low who have bleeding complications require inpatient monitoring and urgent intervention 1

Emergency Treatment Protocol

Initiate combination therapy immediately without waiting for diagnostic confirmation: 1, 2

1. Corticosteroids (start immediately):

   • Methylprednisolone 1g IV daily for 3 days 1
   • Follow with prednisone 1-2 mg/kg/day 1

2. IVIG (for rapid platelet increase):

   • Administer 1 g/kg as a single dose 1
   • IVIG combined with corticosteroids provides the most rapid increase in platelet count when bleeding is present 1
   • May repeat the dose if necessary 1

3. Platelet Transfusion Considerations:

   • Avoid platelet transfusions unless life-threatening bleeding develops (e.g., intracranial hemorrhage) 1, 2
   • For hematuria alone, platelet transfusions are not recommended 2
   • If critical hemorrhage occurs, transfuse platelets in conjunction with IVIG and corticosteroids 1, 2

Monitoring and Supportive Care

• Monitor platelet counts daily - expect increase within 1-2 weeks of starting treatment 3
• Monitor hemoglobin weekly during treatment 4
• Provide folic acid 1 mg daily supplementation 4
• Transfuse RBCs conservatively only if symptomatic anemia develops, targeting hemoglobin 7-8 g/dL 4
• Ensure follow-up with hematology within 24-72 hours 1

Critical Pitfalls to Avoid

Do not delay treatment waiting for bone marrow examination - bone marrow biopsy is not necessary in patients with typical ITP features 1

Do not use anti-D immunoglobulin in this patient with active bleeding and decreased hemoglobin from hematuria, as anti-D is contraindicated when hemoglobin is already decreased due to bleeding 1

Do not normalize platelet counts - the goal is to achieve a platelet count >50 × 10⁹/L to reduce bleeding risk, not to reach normal levels 3

If Bleeding Worsens or Becomes Life-Threatening

If hematuria progresses to more severe bleeding or intracranial hemorrhage develops: 1

• Continue IVIG and corticosteroids
• Consider platelet transfusions every 30 minutes to 8 hours, potentially with continuous IVIG infusion 1
• Consider recombinant factor VIIa (rfVIIa), though thrombosis risk must be weighed 1
• Consider antifibrinolytic agents (tranexamic acid or aminocaproic acid) as adjunct therapy 1
• Emergency splenectomy is a last resort for truly life-threatening bleeding unresponsive to other measures 1

Refractory Disease Considerations

If the patient fails to respond to initial corticosteroids and IVIG after several days: 1

• Add rituximab 375 mg/m² weekly for 3-4 weeks for significant ongoing bleeding 1
• Consider high-dose dexamethasone as an alternative 1
• Splenectomy remains definitive treatment for chronic refractory ITP but should be delayed until other options exhausted 1

Prognostic Factors

This patient is at increased risk for serious morbidity - severe thrombocytopenia with active hemorrhage represents a high-risk scenario requiring aggressive management 5