Victoria’s royal disease

Analysis of the bones of some of her descendants has revealed that Queen Victoria was a carrier of Christmas disease, a severe form of haemophilia.

The hereditary condition has been revealed by genetic analysis of the bones of the wife and children of the last Russian Tsar, Nicholas II.

According to a study published in Science, Tsarina Alexandra, who was a granddaughter of Queen Victoria, carried the genetic mutation, passing it on to two of her children, Crown Prince Alexei and his sister Anastasia.

Genetic defects responsible for haemophilia are located on the X chromosome, which means that women can carry the disease but are rarely sufferers. So while Alexei suffered disease symptoms, Anastasia was simply a carrier.

The haemophilia gene appears to have stemmed from a spontaneous mutation carried by Victoria. Through two of her five daughters (Princess Alice and Princess Beatrice), she passed the mutation to royal houses across the continent, including those in Spain, Germany and Russia. Hence this form of haemophilia became known as the royal disease.

There are two main types of haemophilia, both caused by low levels or complete absence of a blood protein essential for clotting. Patients with haemophilia A lack factor VIII and those with haemophilia B, or Christmas disease, lack factor IX. The UK has about 6,000 haemophilia sufferers: 85 per cent of them have type A and the remainder suffer from type B.

Incidentally, the name Christmas disease has no seasonal connotations. When first identified, in 1952, it was named after Stephen Christmas, a 10-year-old boy with the condition.