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International Journal of Hematology

, Volume 110, Issue 2, pp 197–204 | Cite as

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

  • Masahiro IekoEmail author
  • Mika Yoshida
  • Sumiyoshi Naito
  • Kazumasa Ohmura
  • Nobuhiko Takahashi
Original Article

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.

Keywords

Lupus anticoagulant Prothrombin activity Anti-prothrombin antibodies Anti-factor VIII antibodies 

Notes

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.

Compliance with ethical standards

Conflict of interest

The authors have no conflicts of interest to declare.

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Copyright information

© Japanese Society of Hematology 2019

Authors and Affiliations

  1. 1.Department of Internal Medicine, School of DentistryHealth Sciences University of HokkaidoTobetsuJapan
  2. 2.Division of Clinical LaboratoryHealth Sciences University HospitalTobetsuJapan

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