| Emergent therapy∗ | The use of eligible, first-line, short-acting agents (eg, corticosteroids, IVIG, and intravenous anti-Rho(D) immunoglobulin) to achieve a rapid improvement in platelet count† in the setting of active bleeding |
| Disease-modifying therapy∗ | The use of pharmacotherapy agents‡ (eg, thrombopoietin-receptor agonists§, biologics|, and immunomodulating agents¶) and interventions (eg, splenectomy) to achieve a sustained, durable maintenance response in platelet count to prevent ongoing exacerbations of symptomatic ITP and improve quality of life |
| Emergent therapy∗ | The use of eligible, first-line, short-acting agents (eg, corticosteroids, IVIG, and intravenous anti-Rho(D) immunoglobulin) to achieve a rapid improvement in platelet count† in the setting of active bleeding |
| Disease-modifying therapy∗ | The use of pharmacotherapy agents‡ (eg, thrombopoietin-receptor agonists§, biologics|, and immunomodulating agents¶) and interventions (eg, splenectomy) to achieve a sustained, durable maintenance response in platelet count to prevent ongoing exacerbations of symptomatic ITP and improve quality of life |
Medications assumed to be administered at an effective dose and for an adequate duration.
Bleeding is rare with a platelet count of >20× 109/L to 30 × 109/L, therefore the platelet count is used as a surrogate of treatment response, which eliminates bleeding in the emergent context.
Pharmacotherapy agents with different mechanisms of action are currently in development and in clinical trials. Examples of currently available mechanisms of actions are listed here.
Thrombopoietin-receptor agonists: for example, eltrombopag and romiplostim.
Biologics: for example, rituximab and bortezomib.
Immunomodulating agents: for example, sirolimus, mycophenolate mofetil, azathioprine, and hydroxychloroquine.