Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding

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Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding. / Ajzner, E; Balogh, I; Haramura, G; Boda, Z; Kalmar, K; Pfliegler, G; Dahlbäck, Björn; Muszbek, L.

In: Journal of Thrombosis and Haemostasis, Vol. 1, No. 5, 2003, p. 943-949.

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Ajzner, E ; Balogh, I ; Haramura, G ; Boda, Z ; Kalmar, K ; Pfliegler, G ; Dahlbäck, Björn ; Muszbek, L. / Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding. In: Journal of Thrombosis and Haemostasis. 2003 ; Vol. 1, No. 5. pp. 943-949.

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TY - JOUR

T1 - Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding

AU - Ajzner, E

AU - Balogh, I

AU - Haramura, G

AU - Boda, Z

AU - Kalmar, K

AU - Pfliegler, G

AU - Dahlbäck, Björn

AU - Muszbek, L

PY - 2003

Y1 - 2003

N2 - Development of autoantibody against coagulation factor V (FV) is a rare clinical condition with hemorrhagic complications of varying severity. The aim of this study was to establish the pathomechanism of an acquired FV deficiency and characterize the FV inhibitor responsible for the clinical symptoms. A 78-year-old female was admitted to hospital with severe gastrointestinal bleeding. General clotting tests and determination of clotting factors were performed by standard methods. FV antigen and FV containing immune complexes were measured by ELISA. The FV molecule was investigated by Western blotting and by sequencing the f5 gene. The binding of patient's IgG to FV and activated FV (FVa) was demonstrated in an ELISA system and its effect on the procoagulant activity of FVa was tested in clotting tests and in a chromogenic prothrombinase assay. Localization of the epitope for the antibody was performed by blocking ELISA. FV activity was severely suppressed both in plasma and platelets. FV antigen levels were normal by ELISA using polyclonal anti-FV antibody or monoclonal antibody against the connecting region of FV, but depressed when HV1 monoclonal antibody against the C2 domain in the FV light-chain was used as capture antibody. The FV molecule was found intact. An IgG reacting with both FV and FVa was present in the patient's plasma and its binding to FV was inhibited by HV1 antibody. FV-containing immune complexes were detected in the patient's plasma and platelet lysate. The patient's IgG inhibited the procoagulant function of FVa. An anti-FV IgG was present in the patient's plasma and platelets. The autoantibody reacted with an epitope in the C2 domain of FV light chain and neutralized the procoagulant function of FVa.

AB - Development of autoantibody against coagulation factor V (FV) is a rare clinical condition with hemorrhagic complications of varying severity. The aim of this study was to establish the pathomechanism of an acquired FV deficiency and characterize the FV inhibitor responsible for the clinical symptoms. A 78-year-old female was admitted to hospital with severe gastrointestinal bleeding. General clotting tests and determination of clotting factors were performed by standard methods. FV antigen and FV containing immune complexes were measured by ELISA. The FV molecule was investigated by Western blotting and by sequencing the f5 gene. The binding of patient's IgG to FV and activated FV (FVa) was demonstrated in an ELISA system and its effect on the procoagulant activity of FVa was tested in clotting tests and in a chromogenic prothrombinase assay. Localization of the epitope for the antibody was performed by blocking ELISA. FV activity was severely suppressed both in plasma and platelets. FV antigen levels were normal by ELISA using polyclonal anti-FV antibody or monoclonal antibody against the connecting region of FV, but depressed when HV1 monoclonal antibody against the C2 domain in the FV light-chain was used as capture antibody. The FV molecule was found intact. An IgG reacting with both FV and FVa was present in the patient's plasma and its binding to FV was inhibited by HV1 antibody. FV-containing immune complexes were detected in the patient's plasma and platelet lysate. The patient's IgG inhibited the procoagulant function of FVa. An anti-FV IgG was present in the patient's plasma and platelets. The autoantibody reacted with an epitope in the C2 domain of FV light chain and neutralized the procoagulant function of FVa.

KW - hemorrhagic diathesis

KW - factor V

KW - factor V inhibitor

KW - platelets

U2 - 10.1046/j.1538-7836.2003.00143.x

DO - 10.1046/j.1538-7836.2003.00143.x

M3 - Article

VL - 1

SP - 943

EP - 949

JO - Journal of Thrombosis and Haemostasis

T2 - Journal of Thrombosis and Haemostasis

JF - Journal of Thrombosis and Haemostasis

SN - 1538-7933

IS - 5

ER -