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In the previous post, we talked about the pathophysiology of TTP and clinical features helping us recognize it
Let’s now talk about the management of TTP
I like to think of it in 2 stages. The initial management and prevention of recurrence.
Initial management-
The initial goal in TTP is to replenish ADAMTS13 and stop the microthrombi from forming and propagating. The fastest way to do this is to replace the patient’s plasma with donor plasma that has ADAMTS13.
Hence, we start with plasma exchange or PLEX. PLEX is used in many other diseases such as Guillian Barre or IgM monoclonal gammopathies to remove the offending antibody and the replacement for the removed plasma is albumin. However, in TTP the replacement must be fresh frozen plasma or FFP because the purpose of PLEX is not just to remove the antibody against ADAMTS13 that is possibly causing a problem but to also REPLENISH normal ADAMTS13 to stop the thrombotic and hemolytic process.
Caplacizumab is a novel monoclonal antibody that inhibits the interaction between von Willebrand factor and platelet receptors and prevents the platelets from aggregating further inhibiting thrombosis. Caplacizumab is approved for acute management of TTP in conjunction with PLEX.
Prevention of recurrence-
It helps to remember that the pathophysiology of TTP involves an autoantibody against ADAMTS13 because the goal then is to stop the production of this antibody and to try and put the patient in remission.
Therapeutic options include
Corticosteroids- usually started at 1 mg/kg weight-based dosing and tapered down over a period of several weeks
Rituximab- targets B-cells that produce antibodies
Other options include bortezomib, cyclosporine, vincristine, cyclophosphamide and splenectomy
Recombinant ADAMTS13 is available but only approved for congenital TTP.
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