Haemophilia in European royalty

Queen Victoria's family members with haemophilia
Inheritance by female carriers

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, also suffered from the disease. For this reason, haemophilia was once popularly called "the royal disease". Tests on the remains of the Romanov imperial family show that the specific form of haemophilia passed down by Queen Victoria was probably the relatively rare Haemophilia B.[1]

The sex-linked X chromosome disorder manifests almost exclusively in males, even though the genetic mutation causing the disorder is located on the X chromosome and can be inherited from the mother by male children or from either mother or father by female children. This is because the trait is recessive, meaning that only one correctly-functioning copy of the blood clotting factor gene is necessary for normal clotting. Females have two X chromosomes, and hence redundant copies of the blood clotting factor gene located on them. A female who inherits a mutated copy on one X chromosome has also inherited a second X chromosome from the other parent that is likely to carry a non-mutated copy of the gene, capable of directing appropriate clotting. Such a female, with normal clotting but possessing a single mutated copy of the gene, is called a carrier. Males only possess a single X chromosome, inherited from their mother, having received a Y chromosome from their father instead of a second X. If their sole X chromosome contains the haemophilia mutation they possess no second copy to provide for normal function, as in carrier females. Each child of a carrier will have a 50% chance of inheriting their mother's mutation, of being a haemophiliac (sons) or carrier (daughters). The daughter of a male haemophiliac will always inherit his mutation, while a son cannot ever inherit it. A female will only be affected with haemophilia in the rare circumstance that she inherits mutated X chromosomes from both a haemophiliac father and a carrier mother. No case of such double inheritance is known among Queen Victoria's descendants.

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's appears to have been a spontaneous or de novo mutation and she is usually considered the source of the disease in modern cases of haemophilia among her descendants. 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 she was a carrier, but among her children only Victoria received the mutated copy. The rate of spontaneous mutation is known to increase with paternal age, and Victoria's father was 51 at her birth.

Queen Victoria's eldest daughter, Victoria, Princess Royal, apparently escaped the haemophilia gene as it did not appear in any of her matrilineal 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. Queen Victoria's sons Edward, Alfred, and Arthur were not haemophiliacs; however, her daughters Alice and Beatrice were confirmed carriers of the gene, and Victoria's son Leopold was a sufferer of haemophilia, making his daughter Princess Alice, Countess of Athlone a carrier as well.

Queen Victoria family treeEdit


Victoria, German Empress (1840–1901) Issue: Wilhelm II of Germany, Charlotte, Duchess of Saxe-Meiningen, Prince Henry of Prussia, Prince Sigismund of Prussia, Viktoria, Princess Adolf of Schaumburg-Lippe, Prince Waldemar of Prussia, Sophia, Queen of the Hellenes, Margaret, Landgravine of Hesse-Kassel

Edward VII, King of the United Kingdom (1841–1910) Issue: Prince Albert Victor, Duke of Clarence and Avondale, George V, Louise, Princess Royal, Princess Victoria, Maud, Queen of Norway, Prince Alexander John

Princess Alice, Grand Duchess of Hesse and by Rhine (1843–1878) Issue: Victoria Mountbatten, Marchioness of Milford Haven, Grand Duchess Elizabeth Feodorovna of Russia, Irene, Princess Henry of Prussia, Ernest Louis, Grand Duke of Hesse and by Rhine, Prince Friedrich, Alexandra Feodorovna, Empress of Russia, Princess Marie of Hesse and by Rhine

Prince Alfred, Duke of Saxe-Coburg and Gotha (1844–1900) Issue: Alfred, Hereditary Prince of Saxe-Coburg and Gotha, Marie, Queen of Romania, Victoria Melita, Grand Duchess of Russia, Alexandra, Princess of Hohenlohe-Langenburg, Princess Beatrice, Duchess of Galliera

Helena, Princess Christian of Schleswig-Holstein (1846–1923) Issue: Prince Christian Victor, Albert, Duke of Schleswig-Holstein, Princess Helena Victoria, Princess Marie Louise, Prince Harald

Princess Louise, Duchess of Argyll (1848–1939) No issue

Prince Arthur, Duke of Connaught and Strathearn (1850–1942) Issue: Margaret, Crown Princess of Sweden, Prince Arthur of Connaught, Princess Patricia, Lady Ramsay

Prince Leopold, Duke of Albany (1853–1884) Issue: Princess Alice, Countess of Athlone, Charles Edward, Duke of Saxe-Coburg and Gotha

Beatrice, Princess Henry of Battenberg (1857–1944) Issue: Alexander Mountbatten, 1st Marquess of Carisbrooke, Victoria Eugenie, Queen of Spain, Lord Leopold Mountbatten, Prince Maurice of Battenberg

Princess AliceEdit

Alice (1843–1878), Victoria's third child, and wife of the future Grand Duke Louis IV of Hesse and by Rhine (1837–1892), passed it on to at least three of her children: Irene, Friedrich, and Alix.

Prince LeopoldEdit

Leopold (1853–1884), Victoria's eighth child, was the first member of the family to manifest haemophilia; he died at age 30 from bleeding after a minor fall,[5] only two years after marrying Princess Helena of Waldeck and Pyrmont. (1861–1922)

He passed the gene on to his only daughter, as all of the daughters of a haemophiliac father would inherit the gene:

Alice's younger son Prince Maurice of Teck died in infancy, so it is not known if he was a sufferer. Her daughter Lady May Abel Smith (1906–1994), Leopold's granddaughter, has living descendants none of whom has been known to have or to transmit haemophilia.

Leopold's posthumous son, Charles Edward (1884–1954), was unaffected, as a father cannot pass the gene to a son.

Princess BeatriceEdit

Beatrice (1857–1944), Victoria's ninth and last child, and wife of Prince Henry of Battenberg (1858–1896) 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.

Chronological orderEdit

Queen Victoria died in 1901 and lived to see her youngest son and a grandson die from the disease. The gene can be passed down the female line without a haemophiliac son being born, but as the family line continues and no haemophiliac sons are born, it becomes less likely that a certain ancestor had the gene and passed it on through the female line.

Men who died of haemophilia in order of death
# 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 Lord Leopold Mountbatten 23-Apr-1922 grandson
5 Prince Rupert of Teck 15-Apr-1928 great grandson
6 Infante Gonzalo of Spain 13-Aug-1934 great grandson
7 Alfonso, Prince of Asturias 6-Sep-1938 great grandson
8 Prince Waldemar of Prussia 2-May-1945 great grandson

Type of haemophilia discoveredEdit

Because the last known descendant of Queen Victoria with haemophilia 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. Specifically, they found a single-nucleotide change in the gene for clotting Factor IX that causes incorrect RNA splicing and produces a truncated, nonfunctional protein.[6]


  1. ^ a b "Case Closed: Famous Royals Suffered from Hemophilia". Archived April 12, 2010, at the Wayback Machine Michael Price, ScienceNOW, October 9, 2009.
  2. ^ "Hemophilia B". Retrieved 2007-11-21.
  3. ^ Ian Vorres, The Last Grand Duchess, 1965 p. 115.
  4. ^ Massey, Nicholas and Alexandra, 1967
  5. ^ Aronova-Tiuntseva, Yelena; Clyde Freeman Herreid (2003). "Hemophilia: "The Royal Disease"" (PDF). Science Cases: 1–7. Archived from the original (PDF) on 28 September 2013. Retrieved 23 March 2013.
  6. ^ Rogaev EI, Grigorenko AP, Faskhutdinova G, Kittler EL, Moliaka YK (November 2009). "Genotype analysis identifies the cause of the "royal disease"". Science. 326 (5954): 817. Bibcode:2009Sci...326..817R. doi:10.1126/science.1180660. PMID 19815722.

References and external linksEdit