Factor VII Activity and Antigen in Haemophilia B Variants

M G Mazzucconi; R M Bertina; D Romoli; M Orlando; G Avvisati; G Mariani

doi:10.1055/s-0038-1650001

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1980; 43(01): 016-019
DOI: 10.1055/s-0038-1650001

Original Article

Schattauer GmbH Stuttgart

Factor VII Activity and Antigen in Haemophilia B Variants

M G Mazzucconi

The Institute of Haematology, University of Rome, Italy; Haemostasis and Thrombosis Research Unit, Academisch Ziekenhuis, Leiden, Holland; Istituto Superiore di Sanità, Roma, Italy

,

R M Bertina

The Institute of Haematology, University of Rome, Italy; Haemostasis and Thrombosis Research Unit, Academisch Ziekenhuis, Leiden, Holland; Istituto Superiore di Sanità, Roma, Italy

,

D Romoli

The Institute of Haematology, University of Rome, Italy; Haemostasis and Thrombosis Research Unit, Academisch Ziekenhuis, Leiden, Holland; Istituto Superiore di Sanità, Roma, Italy

,

M Orlando

The Institute of Haematology, University of Rome, Italy; Haemostasis and Thrombosis Research Unit, Academisch Ziekenhuis, Leiden, Holland; Istituto Superiore di Sanità, Roma, Italy

,

G Avvisati

The Institute of Haematology, University of Rome, Italy; Haemostasis and Thrombosis Research Unit, Academisch Ziekenhuis, Leiden, Holland; Istituto Superiore di Sanità, Roma, Italy

,

G Mariani

The Institute of Haematology, University of Rome, Italy; Haemostasis and Thrombosis Research Unit, Academisch Ziekenhuis, Leiden, Holland; Istituto Superiore di Sanità, Roma, Italy

› Author Affiliations

Abstract

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Summary

Twenty three patients belonging to 18 different pedigrees of Haemophilia B were studied with regard to ox-brain prothrombin time and its correlation to factor VII.

Eleven among them were B-negative (no detectable factor IX antigen), five were B-reduced (factor IX antigen detectable but below the normal values) and seven were B-positive (normal levels of factor IX antigen).

Ox-brain prothrombin time was found prolonged (≥ x̄ + 2.5 SD:99% confidence limits) in nine patients. Factor VII Activity (VII: C) was found reduced in 1/11 B-negative, in 2/5 B-reduced and in 4/7 B-positive patients. Factor VII Antigen (VII: Ag) was found normal in all but one patient.

The ratio VII:C/VII:Ag was abnormal in eight patients independently from the variant of Haemophilia B. The underlying defect which causes the prolongation of Ox-brain prothrombin time due to factor VII: C mild deficiency is heterogeneous. Age, a mild Vitamin K deficiency, the presence of an inhibitor of Factor VII activation and other unknown causes, may be responsible for this pattern.

Keywords

Factor VII antigen - Prothrombin time - Haemophilia B variants - Haemophilia B_M

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References

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