What is the optimal management of a patient with refractory TTP who has had poor response to plasma exchange, steroids, rituximab, especially when caplacizumab is unavailable?

Caplacizumab would obviously be the best thing right now, but if unavailable, there are some data with cyclosporine as well as bortezomib and cyclophosphamide to help with more refractory disease. These measures, unfortunately though, will take some time to have an effect. Intensification of plasma exchange (twice daily, larger volumes) is often discussed and considered, but never shown to clearly be helpful. Refractory disease with a rising LDH and stable or decreasing platelet count is very concerning and ominous. Being aware of the potential for end-organ injury (especially cardiac) is important.