The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

Haemophilia - Tập 10 Số 5 - Trang 593-628 - 2004
Paula Bolton‐Maggs1, David J. Perry2, Elizabeth Chalmers3, L. A. Parapia4, J Wilde5, Michael D. Williams6, Peter W. Collins7, Steve Kitchen8, Gerard Dolan9, Andrew Mumford10
1Department of Clinical Haematology, Manchester Royal Infirmary, Manchester
2Haemophilia Centre and Haemostasis Unit, Royal Free and University College Medical School, London
3Department of Haematology, Royal Hospital for Sick Children, Yorkhill NHS Trust, Glasgow
4Haemophilia Centre, Bradford Royal Infirmary, Bradford
5Department of Haematology, University Hospital Birmingham, Edgbaston, Birmingham, West Midlands
6Department of Clinical and Laboratory Haematology, Birmingham Children's Hospital, Birmingham
7Arthur Bloom Haemophilia Centre, University Hospital of Wales, Heath Park, Cardiff
8Department of Coagulation, Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield
9Department of Haematology, University Hospital, Queens Medical Centre, Nottingham
10Bristol Haemophilia Centre, Bristol Haematology and Oncology Centre, Bristol, UK

Tóm tắt

Summary.  The rare coagulation disorders are heritable abnormalities of haemostasis that may present significant difficulties in diagnosis and management. This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K‐dependent factors, FXI and FXIII. Based on both collective clinical experience and the literature, guidelines for management of bleeding complications are suggested with specific advice for surgery, spontaneous bleeding, management of pregnancy and the neonate. We have chosen to include a section on Ehlers‐Danlos Syndrome because haematologists may be consulted about bleeding manifestations in such patients.

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