Haemophilia in European royalty

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Haemophilia figured prominently in the history of European royalty in the 19th and 20th centuries. Queen Victoria, through two of her five daughters (Princess Alice of the United Kingdom and Princess Beatrice of the United Kingdom), passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia. Victoria's son Leopold suffered from the disease. For this reason, haemophilia was once popularly called "the royal disease".

The sex-linked, X chromosome disorder manifests almost entirely in males, although the gene for the disorder is inherited from the mother. However females carrying the faulty X chromosome can pass the chromosome to their descendents. Expression of the disorder is more common in males due to the fact that females have two X chromosomes while the male only has one. If a male's X chromosome is defective, there is not another to mask the disorder. In about 30% of cases, however, there is no family history of the disorder and the condition is the result of a spontaneous gene mutation. [1]

Victoria appears to have been a spontaneous or de novo mutation, and is considered the source of this line of the disease. Her mother, Victoria, was not known to have a family history of the disease. Descendants of Victoria's maternal half-sister, Feodora, are not known to have suffered from the disease. Queen Victoria's father, Edward, was not a haemophiliac and the probability of her mother having had a lover who suffered from haemophilia is minuscule, primarily due to the low life expectancy of 19th century haemophiliacs.

The royal families' history of haemophilia. Those who suffered from or carried haemophilia are enclosed in a box.
The royal families' history of haemophilia. Those who suffered from or carried haemophilia are enclosed in a box.

Queen Victoria's daughter Victoria apparently escaped the haemophilia gene as it did not appear in any of her descendants. Victoria's fifth child, Helena may or may not have been a carrier; two healthy sons survived to adulthood but two other sons died in infancy and her two daughters did not have issue. Victoria's sixth child, Louise, died without issue. Her sons King Edward VII, Alfred, and Arthur were not haemophiliacs.

Three of Victoria's children were unlucky. The disease passed through her daughters Alice and Beatrice and to her son Leopold.

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[edit] Princess Alice

Alice, Victoria's third child, passed it on to at least three of her children:

Princess Victoria of Hesse and by Rhine (later Victoria, Marchioness of Milford Haven), Alice's oldest child and maternal grandmother to Prince Philip, Duke of Edinburgh, apparently did not inherit the mutation. If she did, she does not appear to have passed it on to her descendants. Princess Elizabeth of Hesse and by Rhine (later Grand Duchess Elizabeth Feodorovna of Russia), may or may not have been a carrier. She was childless when killed by the Bolsheviks in 1918. Princess Marie of Hesse and by Rhine, Alice's seventh and last child, may or may not have been a carrier. She died of diphtheria at the age of four.

[edit] Prince Leopold

Leopold, Victoria's eighth child, was a haemophiliac who died from bleeding after a fall. He lived to the age of 30, long enough to pass the gene on to his only daughter:

Alice of Albany's youngest son Prince Maurice of Teck, died in infancy, so it is not known if he was a sufferer. Her daughter Lady May Abel Smith, Leopold's granddaughter, apparently was not a carrier, as the disease has not appeared in her descendants.

[edit] Princess Beatrice

Princess Beatrice (later Princess Henry of Battenberg), Victoria's ninth and last child, passed it on to at least two, if not three, of her children:

[edit] Today

As of today, hemophilia appears to be extinct in the royal houses of Europe. The last male descendant of Victoria to suffer from the disease was Infante Gonzalo (born 1914). Many sons have been born to European royalty since and none is known to have had hemophilia. However, since the hemophilia gene remains hidden in females with only one bad gene, and female descendants of Victoria exist in several royal houses today, there remains a small chance that the disease could appear again, especially among the female-line Spanish descendants of Princess Beatrice.

  • Infanta Beatríz's two sons were not affected by the disease. Beatriz's eldest daughter, Sandra, has two children, a son and daughter. Her son is not affected, and her daughter has two sons, who are apparently unaffected. Beatríz's youngest daughter, Olimpia, had six children; her only son, Paul, died shortly after his fourth birthday. He could have been a possible sufferer. Another daughter, Laura, also died as a child. Her two eldest daughters, Beatrice and Sibilla are both married with children, none of whom, in the case of their sons, appear to be haemophiliacs. Olimpia's youngest daughters are still unmarried, but there is still a chance they could be carriers.
  • Infanta Maria Cristina had four daughters, all potential carriers. Her eldest daughter, Vittoria Eugenie, had a daughter and three sons, the latter all apparently unaffected. The Infanta's second daughter, Giovanna, had only one child, an unaffected son. Her two youngest daughters, Donna Maria Teresa and Donna Anna Sandra, also have only daughters. Of these, only one, Maria Teresa's second daughter, Isabel, is married, but she also has only a daughter. There is a chance the disease may remain in this branch of Princess Beatrice's descendants.

[edit] Notes

  1. ^ Hemophilia B. Retrieved on 2007-11-21.
  2. ^ Massey, Nicholas and Alexandra, 1967
  3. ^ Ian Vorres, The Last Grand Duchess, 1965 p. 115.

[edit] References and external links

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