Frontier and hotspot evolution in Wiskott-Aldrich syndrome: A bibliometric analysis from 2001 to 2021

Background:Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder. Despite our enormous progress in the strategies used to diagnose, treat, and cure WAS, no bibliometric studies have been performed in this research field. This study explored the trends in WAS research through a bibliometric analysis evaluating relevant literature quantitatively and qualitatively. Methods:The literature concerning WAS from 2001 to 2021 was retrieved from the Science Citation Index Expanded (SCI-expanded) of the Web of Science Core Collection database. Acquired data were then visually analyzed using CiteSpace and VOSviewer. Results:2036 papers were included in the final analysis. The annual publication outputs reached its peak in 2013 but declined in recent years. The dominant position of the United States in WAS research was quite obvious. Harvard University (USA), University College London (UK), and Inserm (France) were the three most prolific institutions. Adrian J. Thrasher exerted significant publication impact and made the most notable contributions in the field of WAS. Blood was the most influential journal with the highest publication outputs, and nearly all the top 10 journals and co-cited journals belonged to Q1. Immune dysregulation, thrombocytopenia, syndrome protein deficiency, stem cell, mutation, and diagnosis were the keywords with the strongest citation burst. Conclusion:From 2001 to 2021, the United States was a global leader in the WAS research. Collaboration between countries and institutions is expected to deepen and strengthen in the future. Research hotspots included pathogenesis, clinical manifestations, diagnosis, and therapy. Our results suggest a greater understanding of the mechanistic underpinnings of immune dysfunction in WAS patients, the application of targeted therapies for individual complications, and the development of curative approaches, which will remain research hotspots in the future.