After clinical review it is clear the patient is deteriorating. He needs urgent treatment for highly likely TTP. He has HIV, hepatitis, troponin, amylase, haemolysis screen (LDH/haptoglobin/retics), TFTs, lupus anticoagulant, autoimmune screen and an ADAMTS13 assay requested. CXR and ECG are performed. The renal team are alerted in order to assist with urgent plasma exchange with 1.5 solvent/detergent treated fresh frozen plasma. A gram of IV methylprednisolone is infused. Folic acid is prescribed 5mg/day.
Questions:
- If plasma exchange cannot immediately be arranged are there any alternatives?
- What if the patient continued to deteriorate despite 1.5 plasma exchange/day?
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If plasma exchange is not immediately acailable then large volumes of plasma may be transfused according to the bcsh guidelines. Obviously watch for fluid overload.
If clinical symptoms progress despite plasma exchange at 1.5 then the frequency of PEX maybe increased with addition of stroids and if that does not wirh then add Rituximab