The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation
Top Cited Papers
Open Access
- 2 September 2004
- journal article
- review article
- Published by Wiley in Haemophilia
- Vol. 10 (5) , 593-628
- https://doi.org/10.1111/j.1365-2516.2004.00944.x
Abstract
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.Keywords
This publication has 169 references indexed in Scilit:
- Factor XI deficiencySeminars in Hematology, 2004
- FACTOR XIII DEFICIENCYBritish Journal of Haematology, 1999
- Hereditary Prothrombin Deficiency Presenting as Intracranial Haematoma in InfancyNeuropediatrics, 1999
- Factor XI deficiency in womenAmerican Journal of Hematology, 1999
- Laboratory performance of haemophilia centres in developing countries: 3 years’ experience of the World Federation of Hemophilia External Quality Assessment SchemeHaemophilia, 1998
- Ehlers‐Danlos syndromes: Revised nosology, Villefranche, 1997American Journal of Medical Genetics, 1998
- Clinical manifestations in 28 Italian and Iranian patients with severe factor VII deficiencyHaemophilia, 1997
- Factor XI Activation by Thrombin and Factor XIaSeminars in Thrombosis and Hemostasis, 1993
- Combined Factor V and Factor VIII Deficiency among Non-Ashkenazi JewsNew England Journal of Medicine, 1982