Helicobacter eradication therapy is the first-line therapy for patients with Helicobacter positive idiopathic thrombocytopenic purpura (ITP) in Japan. Indications for treatement in patients with Helicobacter negative, or post-Helicobacter eradicated ITP are platelet counts less than 20×106/l or severe bleeding. The first-line treatment for these patients is corticosteroids. Thrombopoietin receptor agonists (TPO-RAs), rituximab, and splenectomy are second-line treatments for patients with corticosteroid refractory ITP. The choice of a second-line treatment should be determined in consideration of the advantages and disadvantages of each treatment. TPO-RAs are effective in over 80% of patients; however, long-term administration is usually needed. Rituximab treatment ends in four weeks, but its durable response rate is relatively low. The durable response rate of splenectomy is relatively high; however, it causes long-term complications. Effective treatments for patients with ITP who are refractory to second-line treatments have not been established. Some novel drugs are under clinical trials, and a treatment strategy for these patients is expected to be established.
Keywords: Fostamatinib; Idiopathic thrombocytopenic purpura; Rituximab; Thrombopoietin receptor agonists.