Abstract
Autoimmune von Willebrand factor (VWF) deficiency (AiVWFD) caused by anti-VWF autoantibodies is a rare bleeding disorder, whereas “non-immune” acquired von Willebrand syndrome (AVWS) caused by other etiologies is more common. Therefore, only 40 patients with AiVWFD have been identified in Japan through an ongoing nationwide survey on autoimmune coagulation factor deficiencies. This may be due to the inability to efficiently detect anti-VWF antibodies, as anti-VWF antibody testing is not routine. An 80-year-old Japanese woman developed AVWS and experienced bleeding after two separate common colds. She took the same cold medicine each time and recovered spontaneously after discontinuation of the medicine. Severe VWF deficiency normalized each time. Initial immunological tests did not detect anti-VWF autoantibodies, and thus a diagnosis of “non-immune” AVWS of unknown origin was made. However, after 6 years, new ELISA assays using purified VWF proteins detected free anti-VWF autoantibodies, which led to a retrospective diagnosis of AiVWFD. It is probable that the cold medicine (and/or cold virus infection) induced the autoantibodies, as the recurrence and normalization of the same coagulation abnormality and the clinical course (including drug administration and discontinuation) were completely synchronized. If AiVWFD is suspected, highly sensitive autoantibody tests should be performed.
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Data availability
The data that support the findings of this study are available from the corresponding author upon reasonable request.
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Acknowledgements
This study was approved by the institutional review boards of Yamagata University School of Medicine and Gunma University School of Medicine. All procedures were conducted in accordance with the Declaration of Helsinki. The authors are deeply grateful to Dr. Chicako Yokoyama of Yamagata University for providing rat monoclonal anti-VWF antibodies, Dr. Yasuo Magari of Q-may Laboratory Corporation for providing mouse monoclonal anti-VWF antibodies and ICT kits for the detection of anti-VWF antibodies, and Drs. Kenichi Tawara, Hiroaki Shimizu, Takeki Mitsui, and Takayuki Saito of Gunma University for their invaluable assistance in collecting clinical information on the present patient.
Funding
To A.I. from the Japanese Ministry of Education, Culture, Sports, Science and Technology (MEXT; 16K09820), the Japan Agency for Medical Research and Development (AMED; 16ek0109043h0003), and the Japanese Ministry of Health, Labor, and Welfare (MHLW; 21FC1008).
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Y Ogawa, K Yanagisawa, S Kanaya, and H Handa collected clinical data and samples and proofread the manuscript. M Souri and T Osaki carried out experimental examinations and proofread the manuscript. A Ichinose designed the study, wrote, edited, and proofread the manuscript.
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Ogawa, Y., Ichinose, A., Yanagisawa, K. et al. Retrospectively diagnosed autoimmune VWF deficiency in a patient with repeated hemorrhagic events after two common colds. Int J Hematol (2024). https://doi.org/10.1007/s12185-024-03782-4
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DOI: https://doi.org/10.1007/s12185-024-03782-4