Guidelines for Childhood Immune Thrombocytopenia 2022

Abstract

The Platelet Committee of the Japanese Society of Pediatric Hematology/Oncology published the Guidelines for Childhood Immune Thrombocytopenia in 2022. These comprehensive and practical guidelines were developed according to the recommendations of the Medical Information Distribution Service in Japan. They are as follows: 1) idiopathic thrombocytopenic purpura is converted to immune thrombocytopenia (ITP); 2) thrombocytopenia is defined as a platelet count of less than 100×10³/μL; 3) the traditional classification of “acute” and “chronic” ITP is divided into the new nomenclatures of “newly diagnosed”, “persistent”, and “chronic”; and 4) the modified Buchanan’s bleeding score is adopted to stratify bleeding risks. However, we recommend that the management of children with ITP be based on their bleeding scores, regardless of their platelet counts. Additionally, we suggest that the choice of treatment strategy accommodates each patient’s values and preferences, quality of life, and accessibility to health care. We equally recommend corticosteroids and intravenous immunoglobulin as first-line treatments. Thrombopoietin-receptor agonists and rituximab should be used as second-line treatments. Furthermore, it is proposed that splenectomies be considered with caution. The following clinical questions and recommendations were included: vaccination-related ITP, eradication of Helicobacter pylori, post-splenectomy control of infections, vaccination during or after treatments with corticosteroids/rituximab, management of newborn infants born from mothers with ITP, emergency management of severe bleeding, and management of daily activities. We have clearly described the differences in the pathogenesis and ITP treatments between children and adults, and we hope that these guidelines will contribute to the management of childhood ITP and ultimately improve patient well-being.