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Bleeding Disorders

Hereditary Bleeding Disorders

Hereditary bleeding disorders includes haemophilia A and B, Von Willebrand disease 

  1.  Haemophilia

Haemophilia is an inherited, X-linked lifelong bleeding disorder which affects males almost exclusively. Most  frequent haemorrhage involves joints or muscles. Bleeding parttens differ with age: Infants usually bleed into soft tissues or from the mouth but as the boy grows, characterist joint bleeding becomes more common. 

Haemophilia A (Factor VIII deficiency)

  • Is the most common of the hereditary clotting factor deficiencies and are caused by deficiency of factor VIII

  • The inheritance is sex linked but up to 33% of patient have no family history and result from spontaneous mutation 

Clinical Features: spontaneous joint bleeding without injury, prolonged bleeding after injury, spontaneous muscle bleeding, retroperitoneal bleeding, epistaxis and easy bruising. Complication includes arthropathy and disability. 

Haemophilia B (Factor IX deficiency)

  • Is due to deficiency of clotting factor IX

  • Presentation as in Haemophilia A, this is less common 20%.  

Classification of Haemophilia

Haemophilia is classified as mild, moderate or severe according to the levels of circulating factor VIII or IX and indicates the expected frequency of bleeding.  


Classification  of Hemophilia 

Updated on, 27.10.2020


1. STG page number 10-12

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