LAUSR

In 2012, ITP group of Blood Coagulation Abnormalities Research Team with funding from the Ministry of Health, Labour and Welfare Research Grant for Overcoming Intractable Diseases in Japan published a reference guide for management of adult immune thrombocytopenia (ITP) [1]. The reference guide was based on the existing global standards on ITP treatment, as well as research outcomes and expert opinions, and reflected the experience in Japan at that time. A feature of the reference guide was clarification of H. pylori-associated ITP, which is a unique characteristic of ITP cases in Japan. The reference guide also clarified the criteria for starting treatment for ITP, as well as the treatment target and presented a flowchart of treatment, with corticosteroids as first-line treatment, splenectomy as second-line and other treatments as third-line, together with the respective recommendation levels. The basic concepts of management of adult ITP have not changed significantly since that time, and the 2012 reference guide remains adequately effective. However, in the reference guide, thrombopoietin receptor agonists (TPO-RAs) were positioned as third-line treatment, as these products had only recently been approved in Japan. Since then, we have accumulated experience in the use of these drugs, and their long-term efficacy and safety continue to become clear. Furthermore, in 2017, the indications for rituximab, which has been positioned as second-line treatment in Europe and the US, were extended to adult ITP in Japan. Conversely, the number of patients undergoing splenectomy is declining, and there is an increasingly large divergence between decisions made in clinical practice and the reference guide regarding the selection of second-line treatment for patients resistant to corticosteroids. There is also an accumulation of new evidence on ITP treatment, including administration methods for corticosteroids used as first-line treatment, namely high-dose dexamethasone (HD-DEX). For these reasons, this study group decided to examine the latest reports on adult ITP treatment and present a revised version of the reference guide for the management of adult ITP in Japan. The most significant revision is the new recommendation of TPO-RAs, rituximab,b and splenectomy as second-line treatment, in addition to recommending that The Japanese version of this reference guide was published in Jpn J Clin Hematol (Rinsho Ketsueki) 2019 60:877-896.