How do you manage the patient with TTP?

Key Principles

  • Early resuscitation and supportive care
  • Urgent and rapid treatment or transfer to specialist centre (Treat as urgent as aneurysm)
  • Avoidance of platelet transfusion
  • Early plasma exchange with FFP
  • Immunosuppressive therapy
  • Therapy to reduce thrombosis
Initial Resuscitation & Supportive Care
  • ABC approach treating abnormalities as found:
    • 100% oxygen whilst assessing
    • Obtain IV access and perform diagnostic work-up
    • Consider early central line and vascath for plasma exchange
  • May need intubation if significant neurological sequalae
  • Management of seizures using benzodiazepines
  • Monitor urine output and consider RRT if evidence of
  • IV PPI for patients whilst on high dose steroids
  • For management of haemolysis:
    • Transfuse to target of 70g/dL
    • Commence oral folic acid 5mg OD
Specific Management
  • Platelet transfusions contraindicated unless Major haemorrhage:
    • Worsens thrombosis
    • Usually prothrombotic - lines can be performed without
  • Plasma exchange - Mainstay of treatment:
    • Removes the autoantibodies from the patient's circulation, and replaces their plasma with plasma containing normal levels of vWF-CP
    • Ideally instigated within 3-4 hours of diagnosis
    • Using Octaplas (solvent-detergent prepared FFP deficient in ultra-large multimeric vWF)
    • Daily PEx should continue for at least 2 days after platelet recovery (i.e. pits >150 x 109/L)
  • FFP may be given has holding measure whilst awaiting transfer:
    • Not replacement for PLEX
    • Dose 15ml/Kg
  • Immunomodulatory therapy
    • IV Methylprednisolone 1g for 3 days immediately after PLEX
  • Additional therapy (severe or refractory disease)
    • Rituximab (monoclonal antibody against CD20, found on the surface of B cells)
    • MMF
    • Azathioprine
  • Therapy to reduce thrombosis
    • Aspirin 75mg Once plt >50 x 109/L
    • Prophylactic LMWH once plt >50 x 109/L
Referral & Deposition
  • Needs urgent liaison with haematology if suspected
  • Arrange rapid transfer to specialist centre:
    • Always blue light (Agreement with regional ambulance services)
  • New cases - manage on HDU if not on ICU