Factor IX Deventer-Evidence for the Heterogeneity of Hemophilia BM

R M Bertina; I K van der Linden

doi:10.1055/s-0038-1657148

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 1982; 47(02): 136-140
DOI: 10.1055/s-0038-1657148

Original Article

Schattauer GmbH Stuttgart

Factor IX Deventer-Evidence for the Heterogeneity of Hemophilia B_M

Autor*innen

R M Bertina

The Hemostasis and Thrombosis Research Unit, Department of Medicine, Leiden University Hospital, Leiden, The Netherlands
I K van der Linden

The Hemostasis and Thrombosis Research Unit, Department of Medicine, Leiden University Hospital, Leiden, The Netherlands

Abstract

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Summary

Factor IX Deventer was isolated from the plasma of a patient with severe hemophilia B. The patient was classified as B_M because of an abnormal prolongation (2.1 times) of the ox-brain prothrombin time, that could be corrected by addition of antifactor IX serum. Experiments with the isolated factor IX Deventer showed that one of the two peptide bonds involved in the proteolytic activation of factor IX cannot be cleaved by physiological or non-physiological activators (XIa and RVV-X, respectively). Such a defect can explain why the molecule has no procoagulant activity. At present it is not clear why this defect makes factor IX Deventer such an effective inhibitor of the ox-brain prothrombin time. It is proposed that hemophilia B_M is a heterogeneous disorder.

Key words

Hemophilia B_M - Factor IX B_M - Factor IX Deventer - Activation of factor IX B_M

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Referenzen

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