Management of adults with primary immune thrombocytopenia (ITP) has changed dramatically in the past 10 years. New regimens of corticosteroids for first-line treatment have been introduced and are currently being evaluated in a randomized clinical trial. Many patients may not have durable remissions with initial corticosteroid regimens and may require additional, second-line, treatment. For these patients, rituximab has been increasingly used, as it has for other autoimmune disorders, and new thrombopoietin (TPO)-receptor agonists have been developed. Although splenectomy was the first effective and remains the most effective treatment for ITP, inducing durable complete remissions in 66% of patients, rituximab and TPO-receptor agonists are now additional options for second-line treatment. For patients who continue to have severe and symptomatic thrombocytopenia following failure of multiple treatments, including splenectomy and rituximab, the TPO-receptor agonists are effective as third-line treatment for maintaining safe platelets counts to prevent bleeding symptoms in most patients.
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