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Overshoot of FVIII activity in patients with acquired hemophilia A who achieve complete remission

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Abstract

Acquired hemophilia A (AHA) is a rare, life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Immunosuppressive therapy for AHA aims to arrest bleeding by eliminating FVIII inhibitors. Factor VIII activity overshoot after complete remission (CR) has been reported anecdotally, but details remain unclear. We retrospectively analyzed data from 17 patients with AHA who achieved CR under immunosuppressive therapy between 2009 and 2019 at Gunma University Hospital. FVIII activity overshoot was defined as ≥ 150%. All 17 patients had low FVIII activity (median 2.1%; range < 1.0–8.9%) due to FVIII inhibition (median 14.7 BU/mL; range 2.0–234.0) and all achieved CR within a median of 39 (range 19–173) days. Overshoot occurred in 11 (64.7%) patients and maximal FVIII activity reached > 200% in six of them. The median duration from CR to overshoot was 13 (range 0–154) days. The FVIII overshoot was transient (72.7%) or persistent (27.3%). Venous thromboembolism developed as a complication of overshoot in one patient due to iliac vein compression by a massive hematoma. Overshoot of FVIII activity after CR occurs more frequently than previously expected in patients with AHA.

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Acknowledgements

We are grateful to the patients who participated in this study.

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Authors and Affiliations

  1. Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan

    Yoshiyuki Ogawa, Kunio Yanagisawa, Chiaki Naito, Hideki Uchiumi, Takuma Ishizaki, Hiroaki Shimizu & Hiroshi Handa

  2. The Japanese Collaborative Research Group (JCRG) on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labour and Welfare, Yamagata, Japan

    Yoshiyuki Ogawa, Masahiro Ieko & Akitada Ichinose

  3. Hidaka-Kai Shirane Clinic, Numata, Japan

    Hideki Uchiumi

  4. Department of Internal Medicine, National Hospital Organization Takasaki General Medical Center, Takasaki, Japan

    Fumito Gohda

  5. Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, Ishikari-Tonetsu, Japan

    Masahiro Ieko

  6. Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan

    Akitada Ichinose

Authors
  1. Yoshiyuki Ogawa
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  2. Kunio Yanagisawa
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  3. Chiaki Naito
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  4. Hideki Uchiumi
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  5. Takuma Ishizaki
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  6. Hiroaki Shimizu
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  7. Fumito Gohda
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  8. Masahiro Ieko
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  9. Akitada Ichinose
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  10. Hiroshi Handa
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Contributions

YO, AI, MI and HH designed the study. YO, KY, CN, HU, TI, HS, FG, and HH evaluated patients. YO, KY and CN analyzed the data. YO, AI and HH prepared the manuscript, which all the authors reviewed.

Corresponding author

Correspondence to Yoshiyuki Ogawa.

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Ogawa, Y., Yanagisawa, K., Naito, C. et al. Overshoot of FVIII activity in patients with acquired hemophilia A who achieve complete remission. Int J Hematol 111, 544–549 (2020). https://doi.org/10.1007/s12185-020-02823-y

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  • DOI: https://doi.org/10.1007/s12185-020-02823-y

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