What are the clinical signs?

The 3 forms of haemophilia A

Classification is based on plasma activity levels of factor VIII.

Severity Factor VIII activity in plasma Clinical signs
Severe Factor VIII < 1% (< 1 U/dL) Spontaneous serious bleeding, mainly in joints and muscles
Moderate 1% (1 U/dl) = Factor VIII < 5% (5 U/dL) Occasional spontaneous bleeding; serious bleeding in the event of trauma or surgery
Mild 5% (5 U/dL) = Factor VIII = 40% (40 U/dL) Serious bleeding in the event of trauma or surgery

Special features of the severe forms

Severe forms of haemophilia A are associated with the most serious haemorrhagic signs. These can appear after seemingly innocuous trauma that often go unnoticed and such episodes are thus referred to as spontaneous haemorrhages. There is a high risk of haemorrhage in the event of surgery.

Severe haemophilia A occurs in 30 to 40% of cases.

The most common haemorrhagic episodes consist of:

  • haemarthrosis: 70%,
  • subcutaneous or intramuscular haematomas: 10 to 20%,
  • haemorrhaging in the urinary tract, nasal and gastrointestinal mucosa, and intra-abdominal organs: 10 to 20%.


Gastrointestinal and central nervous system haemorrhagic accidents are potentially life-threatening, while others such as bleeding in the orbit, the front of the forearm or the axilla may compromise function. Emergency treatment is required and involves administration of suitable anti-haemophilic products.