What are the main symptoms and signs of haemophilia?
Patients with either form of haemophilia have abnormal haemostasis and so suffer from a tendency to bleed. There are considerable variations in the degree of this bleeding tendency among patients. Very mild forms of the disease are sometimes observed in female carriers, but for practical purposes all severely affected patients are male. The features of haemophilia A depend on the levels of Factor VIII: Levels of less than 1% are associated with frequent and often severe spontaneous bleeding from early life. These patients are particularly prone to certain forms of bleeding: • Bleeding into joints, called haemarthroses, causes pain, redness and swelling of the larger joints (often the knee). Repeated episodes can lead to joint deformity that can be crippling without adequate treatment. • Muscular haematomas, bleeding into muscle, is common, either after relatively minor trauma or intramuscular injections. This should be avoided in these patients. • Deep visceral (internal organs) bleeds