Therapies for managing TTP and their mechanism of action
| Therapy | Mechanism |
|---|---|
| TPE | Removes anti–ADAMTS-13 antibodies, ADAMTS-13 immune complexes, and ULVWF |
| Rituximab | Chimeric monoclonal antibody directed at CD20 on mature B lymphocytes downregulating the immune response |
| Caplacizumab | Bivalent humanized nanobody that attaches to the A1 domain of VWF and inhibits its interaction with the GPIb receptor on platelets, reducing their activity |
| NAC | Adjuvant therapy with a structure similar to disulfide linkages in VWF used to reduce large VWF multimers and inhibit VWF-dependent platelet aggregation |
| Bortezomib | Proteasome inhibitor that inhibits B-lymphocyte antibody production |
| rADA | Replaces circulating ADAMTS-13 and reduces some of the adverse effects associated with fresh frozen plasma |
| Anfibatide | Potent platelet GPIb receptor antagonist derived from snake venom, which inhibits platelet aggregation |