Haemophilia figured prominently in the history of European royalty in the 19th and 20th centuries. Britain's Queen Victoria, through two of her five daughters (Princess Alice and Princess Beatrice), passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia. Victoria's son Prince Leopold, Duke of Albany suffered from the disease. For this reason, haemophilia was once popularly called "the royal disease". Tests of the remains of the Romanov imperial family show that the specific form of haemophilia passed down by Queen Victoria was likely the relatively rare Haemophilia B.[1]
The sex-linked X chromosome disorder manifests almost entirely in males, although the gene for the disorder is located on the X chromosome and may be inherited from either mother or father. Expression of the disorder is much more common in males than in females. This is because, although the trait is recessive, males only inherit one X chromosome, from their mothers. Thus if the haemophilia gene is transmitted on it, there is no possibility for the male to inherit a haemophilia-free gene from his father to mask or dilute the symptoms. By contrast, a female who inherits a gene for haemophilia on one of her X chromosomes will also have inherited a second X chromosome from the other parent which is likely to carry a haemophilia-free gene that would prevent full expression of symptoms.
Females who inherit the gene for Haemophilia A or B from both parents would be expected to manifest full symptoms, similar to those seen in affected males, but this is extremely rare. Despite frequent inter-marriage among royalty, no case of such double inheritance is known among Queen Victoria's descendants. However, it is possible that such cases have gone undocumented because of the potentially higher mortality affected females would likely experience, especially upon onset of menarche.
Although an individual's haemophilia can usually be traced in the ancestry, in about 30% of cases there is no family history of the disorder and the condition is speculated to be the result of spontaneous mutation in an ancestor.[2]
Victoria appears to have been a spontaneous or de novo mutation and is usually considered the source of the disease in modern cases of haemophilia among royalty. Queen Victoria's father, Prince Edward, Duke of Kent, was not a haemophiliac, and the probability of her mother having had a lover who suffered from haemophilia is minuscule given the low life expectancy of 19th century haemophiliacs. Her mother, Victoria, Duchess of Kent, was not known to have a family history of the disease, although it is possible that the mutation began at her conception and was passed down only to Victoria and not to her other children. In the same way, had Queen Victoria herself only had seven children, the mutation would likely be assumed today to have occurred at the conception of Princess Alice, as she was the only known carrier among Victoria and Albert's first seven children.
Queen Victoria's eldest 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. However, her son Leopold was a sufferer of haemophilia and her daughters Alice and Beatrice were confirmed carriers of the gene.
Contents |
|
|||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Alice, Victoria's third child, passed it on to at least three of her children: Friedrich, Irene, and Alix.
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 (his posthumous son being unaffected, as is usual when the gene is carried only by the father):
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, has living descendants none of whom has been known to have or to transmit haemophilia.
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 four children:
No living member of the present or past reigning dynasties of Europe is known to have symptoms of haemophilia or is believed to carry the gene for it. The last descendant of Victoria known to suffer from the disease was Infante Don Gonzalo, born in 1914, although dozens of descendants of Queen Victoria's (including males descended only through females) have been born since 1914. However, because the haemophilia gene usually remains hidden in females who only inherit the gene from one parent, and female descendants of Victoria have left many descendants in royal and noble families, there remains a small chance that the disease could appear again, especially among the female-line Spanish descendants of Princess Beatrice.
At least one modern descendant of Queen Victoria has been diagnosed with haemophilia: Ferdinand Soltmann, the son of Princess Xenia of Hohenlohe-Langenberg, born 2005. Xenia is a male-line descendant of Victoria, but the disease did not come from Xenia's maternal family, the Croÿs. If the disease came from Xenia, there are two possibilities. The first possibility is that it would have had to be inherited from her father, Kraft, Prince of Hohenlohe-Langenberg, a descendant of Victoria through the female line. Kraft had some clotting issues, which led the family to believe he may have been a mild haemophiliac.[6] If Kraft was a haemophiliac, then his daughters Xenia and Cécile were definitely carriers. The second possibility is that Xenia or Ferdinand had a spontaneous mutation, as Victoria herself apparently had.
Xenia is descended from Victoria in two different lines, through Victoria's children Princess Alice of the United Kingdom and Alfred, Duke of Saxe-Coburg and Gotha. Alfred was not a haemophiliac, but Alice was a carrier, and her line of descent to Xenia, and subsequently, Ferdinand, is as follows.
| Queen Victoria | ||||||||||||||
| Princess Alice of the United Kingdom | ||||||||||||||
| Princess Victoria of Hesse and by Rhine | ||||||||||||||
| Princess Alice of Battenberg | ||||||||||||||
| Princess Margarita of Greece and Denmark | ||||||||||||||
| Kraft, 9th Prince of Hohenlohe-Langenburg | ||||||||||||||
| Princess Xenia of Hohenlohe-Langenburg | ||||||||||||||
| Ferdinand Soltmann | ||||||||||||||
Queen Victoria died in 1901 so she lived to see a son and, possibly, a grandson die from the disease. A great-grandson was diagnosed with the disease as well. There is no sign of the disease into later generations, but it has been known to skip several generations.
| # | Name | Death | Relation to Queen Victoria |
|---|---|---|---|
| 1 | Prince Friedrich of Hesse and by Rhine | 29-May-1873 | grandson |
| 2 | The Prince Leopold, Duke of Albany | 28-Mar-1884 | son |
| 3 | Prince Heinrich Friedrich of Prussia | 26-Feb-1904 | great grandson |
| 4 | Prince Maurice of Battenberg (disease unconfirmed) | 27-Oct-1914 | grandson |
| 5 | Lord Leopold Mountbatten | 23-Apr-1922 | grandson |
| 6 | Prince Rupert of Teck | 15-Apr-1928 | great grandson |
| 7 | Infante Gonzalo of Spain | 13-Aug-1934 | great grandson |
| 8 | Alfonso, Prince of Asturias | 6-Sep-1938 | great grandson |
| 9 | Prince Waldemar of Prussia | 2-May-1945 | great grandson |
Because the last known descendent with haemophilia of Queen Victoria's family tree died in the 1940s, the exact type of haemophilia found in this family remained unknown until 2009. Using genetic analysis of the remains of the assassinated Romanov dynasty, and specifically Tsarevich Alexei, Rogaev et al. were able to determine that the "Royal Disease" is actually haemophilia B.[7]