Journal
HAEMOPHILIA
Volume 10, Issue 5, Pages 593-628Publisher
WILEY
DOI: 10.1111/j.1365-2516.2004.00944.x
Keywords
rare coagulation disorders; fibrinogen; prothrombin; FV; FVII; FX; FXI; vitamin K dependent factors; FXIII; Ehlers Danlos syndrome; Factor V plus VIII deficiency; molecular genetics
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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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