Role of lupus anticoagulants in immediate acting inhibitor positivity in congenital haemophilia A patients

SummaryLiposomes of pure phospholipids were used in a modified APTT test system and the role of phosphatidyl serine (PS) in determining the sensitivity of the test system to the presence of lupus anticoagulants was assessed. Six consecutive patients with lupus anticoagulants and seven haemophiliacs with anticoagulants directed at specific coagulation factors, were studied. Increasing the concentration of phospholipid in the test system markedly reduced the sensitivity to lupus anticoagulants but had marginal effect on the specific factor inhibitors. The same effect was achieved when the content of PS alone was increased in a vehicle liposome of constant composition.The results suggest that the lupus anticoagulants can best be detected by a screening method using an APTT test with a reagent of low PS content. The use of a reagent rich in PS will largely abolish the lupus anticoagulant’s effect on the APTT. An approach using the two different types of reagent may facilitate differentiation of lupus inhibitors from other types of anticoagulant.

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Somatic/Germinal Mosaicism of a F8 Promoter Deletion Confounds Clinical Predictions in a Family with Haemophilia A: Key Role of Genotype Quantitation

Thrombosis and Haemostasis ◽

10.1055/s-0038-1623536 ◽

2018 ◽

Vol 118 (03) ◽

pp. 617-620 ◽

Cited By ~ 1

Author(s):

M. Abelleyro ◽

V. Marchione ◽

L. Elhelou ◽

C. Radic ◽

L. Rossetti ◽

...

Keyword(s):

Haemophilia A ◽

Promoter Deletion ◽

Germinal Mosaicism

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Inhibitor development in haemophilia A: the role of von Willebrand factor/factor VIII concentrates

Haemophilia ◽

10.1111/j.1365-2516.2007.01571.x ◽

2007 ◽

Vol 13 ◽

pp. 47-51 ◽

Cited By ~ 6

Author(s):

J. GOUDEMAND

Keyword(s):

Factor Viii ◽

Von Willebrand Factor ◽

Haemophilia A ◽

Inhibitor Development ◽

Factor Viii Concentrates ◽

Von Willebrand ◽

Willebrand Factor

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Role of chromogenic assays in haemophilia A and B diagnosis

Haemophilia ◽

10.1111/hae.13520 ◽

2018 ◽

Vol 24 (4) ◽

pp. 578-583 ◽

Cited By ~ 4

Author(s):

A. E. Bowyer ◽

E. M. Duncan ◽

J. P. Antovic

Keyword(s):

Haemophilia A

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Mechanism of action of β2-glycoprotein I-dependent lupus anticoagulants

Lupus ◽

10.1177/096120339800700206 ◽

1998 ◽

Vol 7 (2_suppl) ◽

pp. 23-28 ◽

Cited By ~ 15

Author(s):

J Amout ◽

J Vermylen

Keyword(s):

Mechanism Of Action ◽

Molecular Basis ◽

Current Knowledge ◽

Indirect Evidence ◽

Integrated Model ◽

Anticardiolipin Antibodies ◽

Pathogenic Role ◽

Lupus Anticoagulants ◽

Glycoprotein I

There is accumulating evidence showing that lupus anticoagulants (LA) are more strongly associated with thrombosis than anticardiolipin antibodies. In addition, indirect evidence has been presented indicating that β2GPI-dependent LA are more strongly associated with thrombosis than prothrombin-dependent LA. From this, one may assume that anti-β2GPl antibodies with LA activity are more pathogenic than anti-β2GPI antibodies without LA activity. Therefore, it is of the utmost importance to understand the molecular basis on which some anti-β2GPI antibodies behave as LA. In this presentation, the current knowledge on the interaction of β2GPI with phospholipids and with anti-β2GPI antibodies is reviewed and an integrated model for the anti-β2GPI - dependent LA activity is proposed with implications for a pathogenic role of these particular antibodies.

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Inhibitors in haemophilia A: the role of VWF/FVIII concentrates - a meeting report

Haemophilia ◽

10.1111/j.1365-2516.2008.01944.x ◽

2009 ◽

Vol 15 (2) ◽

pp. 587-591 ◽

Cited By ~ 4

Author(s):

S. KAVERI ◽

A. GRINGERI ◽

M. HEISEL-KURTH ◽

W. KREUZ

Keyword(s):

Meeting Report ◽

Haemophilia A

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Endocytic receptor for pro-coagulant factor VIII: Relevance to inhibitor formation

Thrombosis and Haemostasis ◽

10.1160/th10-05-0294 ◽

2010 ◽

Vol 104 (12) ◽

pp. 1093-1098 ◽

Cited By ~ 5

Author(s):

Ana-Maria Navarrete ◽

Suryasarathi Dasgupta ◽

Maud Teyssandier ◽

Yohann Repessé ◽

Sandrine Delignat ◽

...

Keyword(s):

Immune System ◽

Factor Viii ◽

Patient Management ◽

Antigen Presenting Cells ◽

Critical Issue ◽

Haemophilia A ◽

Antigen Presenting

SummaryThe immunogenicity of therapeutic factor VIII (FVIII) in patients with haemophilia A remains a critical issue in patient management. This review describes the immunological processes involved in the activation of the immune system against FVIII, with a particular focus on the role of endocytic receptors for the recognition of FVIII by antigen-presenting cells.

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