Synonym(s)
Hemophilia; hemophilia A
HistoryThis section has been translated automatically.
Otto, 1803; Hopff, 1828
DefinitionThis section has been translated automatically.
Hereditary activity deficiency of factor VIII (anti-hemophilic globulin).
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Occurrence/EpidemiologyThis section has been translated automatically.
Proportion of all haemophilia diseases: approx. 75%. Incidence (Federal Republic of Germany): 1/5.000 men.
EtiopathogenesisThis section has been translated automatically.
X-linked mutations of the HEMA Coagulation factor VIII gene (HEMA gene; F8 gene; gene locus: Xq28).
ManifestationThis section has been translated automatically.
Mostly occurring in men. Women are conductors.
Clinical featuresThis section has been translated automatically.
Small area bleeding after minor trauma. Characteristic recurrent bleeding in muscles and joints with subsequent muscular atrophies and contractures, joint stiffening. More frequent spontaneous haemorrhaging in severe haemophilia in adolescence (great urge to move), rare in moderately severe haemophilia.
LaboratoryThis section has been translated automatically.
- Severe haemophilia: factor VIII < 1%. Moderate haemophilia: factor VIII 1-4%. Mild haemophilia: factor VIII 5-25%. Sub-hemophilic haemophilia: factor VIII 25-45%.
- Normal bleeding time; prolonged coagulation time; normal quick-value; reduced prothrombin consumption.
TherapyThis section has been translated automatically.
Substitution of factor VIII.
LiteratureThis section has been translated automatically.
- Hopff (1828) On haemophilia or the hereditary predisposition to fatal bleeding Dissertation, University of Zurich
- Otto JC (1803) An account of an hemorrhagic disposition existing in certain families. Med Repository 6: 1-4
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Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer