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Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan

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Abstract

Patients with lupus anticoagulant (LA), a thrombotic risk factor, along with decreased prothrombin (FII) activity are classified as lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) and occasionally show bleeding symptoms, although this is not essential for diagnosis. We treated 20 cases of LAHPS over a 3-year period. Median FII activity was 20.9% and the anti-prothrombin antibody (anti-II Ab), shown by ELISA findings, was detected in 55%. Bleeding symptoms were observed in 20%, although that finding was not correlated with FII activity or anti-FII Ab quantity. We also observed 21 LA cases with decreased activity of coagulation factors other than FII, which we have designated LAHPS-like syndrome (LLS). Among LLS patients, anti-FII Ab and bleeding symptoms were seen in 47.6% and 14.3%, respectively. Our findings suggest that bleeding in LAHPS and LLS cannot be explained only by FII activity decreased by anti-FII Ab. Low FVIII activity and the anti-FVIII antibody (anti-FVIII Ab) were detected in some LAHPS and LLS patients, making it difficult to distinguish those from acquired hemophilia A cases. Detection of anti-FVIII Ab quantity by ELISA may be useful for accurate determination, as that was not performed in our LAHPS or LLS patients.

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Acknowledgements

We express our appreciation to Hiroko Juraku and Aiko Kumagai for their assistance with the experiments, and Yayoi Ishida for helping to prepare the manuscript.

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Authors and Affiliations

  1. Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, 1757-Kanazawa, Ishikari, Tobetsu, Hokkaido, 061-0293, Japan

    Masahiro Ieko, Kazumasa Ohmura & Nobuhiko Takahashi

  2. Division of Clinical Laboratory, Health Sciences University Hospital, Tobetsu, Japan

    Mika Yoshida & Sumiyoshi Naito

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  1. Masahiro Ieko
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  2. Mika Yoshida
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  3. Sumiyoshi Naito
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  4. Kazumasa Ohmura
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  5. Nobuhiko Takahashi
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Correspondence to Masahiro Ieko.

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Ieko, M., Yoshida, M., Naito, S. et al. Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan. Int J Hematol 110, 197–204 (2019). https://doi.org/10.1007/s12185-019-02674-2

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  • DOI: https://doi.org/10.1007/s12185-019-02674-2

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