Sequence of treatments for adults with primary immune thrombocytopenia.

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.