Von Willebrand’s Syndrome

This is also an inherited disorder, and is practically identical to haemophilia. It occurs about once in 150,000 persons. Bleeding occurs from mucosal surfaces, particularly the nose and gastrointestinal tract. Excessive menstrual bleeding is also common. Pregnancy may be hazardous. The chief difference is that after infusion with Factor VIII, the level rapidly rises, and is maintained at the high level for 24 – 36 hours.

Hereditary Haemorrhagic Telangiectasis. This is another rare inherited disorder. Telangiectases (prominent capillary blood vessels) occur in the nasal lining, on the tongue, lips, face and the alimentary system. They increase in size with time. Although they are seldom serious in youth, with time they may cause serious blood loss and anaemia.

Continuous nose bleeding may become a problem in later years, necessitating the continual use of iron therapy. There is no cure. Nasal lesions must not be cauterised (a common treatment for recurring nosebleed in normal patients). Therapy is similar to that of haemophilia. Sometimes female hormone is given, as this may produce a protective layer over the lesions.