Clinical Practice Guidelines for Childhood Immune Thrombocytopenia 2022 from the Japanese Society of Pediatric Hematology/Oncology

Abstract

The Platelet Committee of the Japanese Society of Pediatric Hematology/Oncology has updated the Consensus Guidelines for Diagnosis and Treatment of Childhood Idiopathic Thrombocytopenic Purpura published in 2004. The present guidelines were developed in accordance with the recommendations of the Medical Information Distribution Service in Japan and are thoroughly evidence-based. We have changed the following: (1) idiopathic thrombocytopenic purpura is now called immune thrombocytopenia (ITP); (2) thrombocytopenia is defined as having a platelet count of less than 100×10³/μL; (3) the traditional distinction of “acute” and “chronic” ITP is divided into new nomenclatures, “newly diagnosed”, “persistent”, and “chronic”; (4) the modified Buchanan’s bleeding score is adopted to classify bleeding risks. We recommend that management strategies for children with ITP should be determined on the basis of their bleeding scores, regardless of their platelet counts. In addition, treatment options should be suggested considering each patient’s values and preferences, quality of life, and access to health care. Corticosteroids and intravenous immunoglobulin are equally recommended as first-line treatments. Thrombopoietin-receptor agonists and rituximab are indicated as second-line treatments. Although splenectomy has been considered as an established treatment, we want to recommend limited use of splenectomy, because splenectomy is invasive. We also included the following clinical issues and recommendations: vaccination-related ITP, eradication of Helicobacter pylori, post-splenectomy control of infection, vaccination during or after treatments with corticosteroids/rituximab, management of newborns born to mothers with ITP, emergency management of severe bleeding, and daily activities. Details of the guidelines will be published as a book. We hope that these guidelines will improve the management of pediatric ITP, and consequently, the patients’ well-being.