Retrospectively diagnosed autoimmune VWF deficiency in a patient with repeated hemorrhagic events after two common colds.

Autor: Ogawa Y; Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan.; The Japanese Collaborative Research Group (JCRG) On Autoimmune Acquired Coagulation Factor Deficiencies Supported By the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan., Ichinose A; The Japanese Collaborative Research Group (JCRG) On Autoimmune Acquired Coagulation Factor Deficiencies Supported By the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan. aichinos@med.id.yamagata-u.ac.jp.; Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, 2-2-2 Iida-Nishi, Yamagata, 990-9585, Japan. aichinos@med.id.yamagata-u.ac.jp., Yanagisawa K; Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan., Souri M; The Japanese Collaborative Research Group (JCRG) On Autoimmune Acquired Coagulation Factor Deficiencies Supported By the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan.; Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, 2-2-2 Iida-Nishi, Yamagata, 990-9585, Japan.; Department of Public Health and Hygiene, Yamagata University Graduate School of Medical Science, Yamagata, Japan., Osaki T; The Japanese Collaborative Research Group (JCRG) On Autoimmune Acquired Coagulation Factor Deficiencies Supported By the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan.; Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, 2-2-2 Iida-Nishi, Yamagata, 990-9585, Japan.; Department of Public Health and Hygiene, Yamagata University Graduate School of Medical Science, Yamagata, Japan., Kanaya S; Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan., Handa H; Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan.
Jazyk: angličtina
Zdroj: International journal of hematology [Int J Hematol] 2024 Sep; Vol. 120 (3), pp. 375-381. Date of Electronic Publication: 2024 May 18.
DOI: 10.1007/s12185-024-03782-4
Abstrakt: 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.
(© 2024. Japanese Society of Hematology.)
Databáze: MEDLINE
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