Overview of definitions and therapeutic options
| Clinical remission . | Biological (ADAMTS13) remission . |
|---|---|
| Definition | |
| Clinical response | Partial: ADAMTS13 activity ≥20% to <LLN |
| • Platelet count ≥150 × 109/L | Complete: ADAMTS13 activity ≥LLN |
| • LDH <1.5 times upper limit of normal | |
| • No new or progressive ischemic organ injury with either (1) no temporizing agents for 30 days, or (2) ADAMTS13 remission | |
| Therapeutics | |
| Temporizing agents for clinical response | Disease-modifying agents for biological remission |
| • Plasma exchange | • Plasma exchange |
| • Caplacizumab | • Steroids |
| • Other anti-VWF therapeutics* | • Rituximab |
| • Recombinant ADAMTS13* | • Other immunomodulating agents |
| Clinical remission . | Biological (ADAMTS13) remission . |
|---|---|
| Definition | |
| Clinical response | Partial: ADAMTS13 activity ≥20% to <LLN |
| • Platelet count ≥150 × 109/L | Complete: ADAMTS13 activity ≥LLN |
| • LDH <1.5 times upper limit of normal | |
| • No new or progressive ischemic organ injury with either (1) no temporizing agents for 30 days, or (2) ADAMTS13 remission | |
| Therapeutics | |
| Temporizing agents for clinical response | Disease-modifying agents for biological remission |
| • Plasma exchange | • Plasma exchange |
| • Caplacizumab | • Steroids |
| • Other anti-VWF therapeutics* | • Rituximab |
| • Recombinant ADAMTS13* | • Other immunomodulating agents |
LDH, lactate dehydrogenase; LLN, lower limit of normal.
In development.