By ULY CLINIC

​

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 

​