Reference guide for the treatment of adult idiopathic thrombocytopenic purpura

The treatment strategy of adult idiopathic thrombocytopenic purpura (ITP) in Japan had consisted of corticosteroids as the first-line option, splenectomy as the second-line option, and others as the third-line option, respectively, for a long time. However, thrombopoietin receptor agonists (TPO-RAs) have been widely adopted recently for corticosteroid-resistant ITP, and indications for rituximab have been extended to adult ITP in Japan, suggesting that ITP treatment is dramatically changing. In the "Reference guide for adult ITP treatments in Japan" revised in 2019, TPO-RAs and rituximab are equally recommended as second-line treatments alongside splenectomy. It is suggested to select from among the second-line treatments with careful consideration of not only their advantages and disadvantages but also aspects of the condition and situation of each patient such as any comorbidities or lifestyle factors. Moreover, since multiple effective therapeutic options are now available as second-line options, it is preferable to consider an early transition to second-line treatments for corticosteroid-refractory/dependent ITP patients.