Immune Thrombocytopenia (ITP) occurs in 2-4:100,000 adults and results in variable bleeding symptoms and thrombocytopenia. In the last decade changes in our understanding of the pathophysiology of the disorder have led to publication of new guidelines for the diagnosis and management of ITP, and standards for terminology. Current evidence supports alternatives to splenectomy for second line management of patients with persistently low platelet counts and bleeding. Long-term follow up data suggest both efficacy and safety, in particular for the thrombopoietin receptor agonists (TPO-RA) and the occurrence of late remissions. Follow up of patients who have undergone splenectomy for ITP reveals significant potential risks that should be discussed with patients and may influence clinician and patient choice of second line therapy. Novel therapeutics are in development to address ongoing treatment gaps.
- Submitted March 14, 2017.
- Accepted April 10, 2017.
- Copyright © 2017 American Society of Hematology
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