Haemophilia in European royalty
Encyclopedia
Haemophilia
Haemophilia
Haemophilia is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation, which is used to stop bleeding when a blood vessel is broken. Haemophilia A is the most common form of the disorder, present in about 1 in 5,000–10,000 male births...

 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
Princess Alice of the United Kingdom
The Princess Alice was a member of the British royal family, the third child and second daughter of Queen Victoria and Prince Albert of Saxe-Coburg and Gotha.Alice's education was devised by Albert's close friend and adviser, Baron Stockmar...

 and Princess Beatrice
Princess Beatrice of the United Kingdom
The Princess Beatrice was a member of the British Royal Family. She was the fifth daughter and youngest child of Queen Victoria and Prince Albert. Juan Carlos, King of Spain, is her great-grandson...

), passed the mutation to various royal houses across the continent, including the royal families of Spain
House of Bourbon
The House of Bourbon is a European royal house, a branch of the Capetian dynasty . Bourbon kings first ruled Navarre and France in the 16th century. By the 18th century, members of the Bourbon dynasty also held thrones in Spain, Naples, Sicily, and Parma...

, Germany and Russia
Romanov
The House of Romanov was the second and last imperial dynasty to rule over Russia, reigning from 1613 until the February Revolution abolished the crown in 1917...

. Victoria's son Prince Leopold, Duke of Albany
Prince Leopold, Duke of Albany
The Prince Leopold, Duke of Albany was the eighth child and fourth son of Queen Victoria and Prince Albert of Saxe-Coburg and Gotha. Leopold was later created Duke of Albany, Earl of Clarence, and Baron Arklow...

 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
Haemophilia B
Haemophilia B is a blood clotting disorder caused by a mutation of the Factor IX gene, leading to a deficiency of Factor IX. It is the second most common form of haemophilia, rarer than haemophilia A. It is sometimes called Christmas disease after Stephen Christmas, the first patient described...

.

The sex-linked X chromosome
X chromosome
The X chromosome is one of the two sex-determining chromosomes in many animal species, including mammals and is common in both males and females. It is a part of the XY sex-determination system and X0 sex-determination system...

 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
Trait (biology)
A trait is a distinct variant of a phenotypic character of an organism that may be inherited, environmentally determined or be a combination of the two...

 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
Menarche
Menarche is the first menstrual cycle, or first menstrual bleeding, in female human beings. From both social and medical perspectives it is often considered the central event of female puberty, as it signals the possibility of fertility....

.

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
Mutation
In molecular biology and genetics, mutations are changes in a genomic sequence: the DNA sequence of a cell's genome or the DNA or RNA sequence of a virus. They can be defined as sudden and spontaneous changes in the cell. Mutations are caused by radiation, viruses, transposons and mutagenic...

 in an ancestor.

Victoria appears to have been a spontaneous or de novo mutation
Mutation
In molecular biology and genetics, mutations are changes in a genomic sequence: the DNA sequence of a cell's genome or the DNA or RNA sequence of a virus. They can be defined as sudden and spontaneous changes in the cell. Mutations are caused by radiation, viruses, transposons and mutagenic...

 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
Prince Edward Augustus, Duke of Kent and Strathearn
The Prince Edward, Duke of Kent and Strathearn was a member of the British Royal Family, the fourth son of King George III and the father of Queen Victoria...

, 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
Princess Victoria of Saxe-Coburg-Saalfeld
Princess Victoria of Saxe-Coburg-Saalfeld was the mother of Queen Victoria of the United Kingdom.-Early life:...

, 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
Victoria, Princess Royal
The Princess Victoria, Princess Royal was the eldest child of Queen Victoria of the United Kingdom and Prince Albert. She was created Princess Royal of the United Kingdom in 1841. She became German Empress and Queen of Prussia by marriage to German Emperor Frederick III...

, apparently escaped the haemophilia gene as it did not appear in any of her descendants. Victoria's fifth child, Helena
Princess Helena of the United Kingdom
Princess Helena was a member of the British Royal Family, the third daughter and fifth child of Queen Victoria and Prince Albert....

, 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
Princess Louise, Duchess of Argyll
The Princess Louise was a member of the British Royal Family, the sixth child and fourth daughter of Queen Victoria and her husband, Albert, Prince Consort.Louise's early life was spent moving between the various royal residences in the...

, died without issue. Her sons King Edward VII
Edward VII of the United Kingdom
Edward VII was King of the United Kingdom and the British Dominions and Emperor of India from 22 January 1901 until his death in 1910...

, Alfred
Alfred, Duke of Saxe-Coburg and Gotha
Alfred, Duke of Saxe-Coburg and Gotha was the third Duke of Saxe-Coburg and Gotha, and reigned from 1893 to 1900. He was also a member of the British Royal Family, the second son and fourth child of Queen Victoria and Prince Albert of Saxe-Coburg and Gotha...

, and Arthur
Prince Arthur, Duke of Connaught and Strathearn
Prince Arthur, Duke of Connaught and Strathearn was a member of the shared British and Saxe-Coburg and Gotha royal family who served as the Governor General of Canada, the 10th since Canadian Confederation.Born the seventh child and third son of Queen Victoria and Prince Albert of Saxe-Coburg and...

 were not haemophiliacs. However, her son Leopold was a sufferer of haemophilia and her daughters Alice and Beatrice were confirmed carrier
Carrier
Carrier may refer to:- Science :* Carrier wave, a waveform suitable for modulation by an information-bearing signal* Charge carrier, an unbound particle carrying an electric charge* a mathematical Set over which an algebraic structure is defined...

s of the gene.

Princess Alice

Alice, Victoria's third child, passed it on to at least three of her children: Friedrich, Irene, and Alix.
  • Prince Friedrich of Hesse and by Rhine
    Prince Friedrich of Hesse and by Rhine
    Prince Friedrich of Hesse and by Rhine , , was the haemophiliac second son of Ludwig IV, Grand Duke of Hesse and by Rhine and Princess Alice of the United Kingdom, thus he is a grandson of Queen Victoria...

     (known in the family as "Frittie"). Died before his third birthday of bleeding on the brain resulting from a fall from a third-storey window (which would almost certainly have been fatal even if he had not had haemophilia).

  • Princess Irene of Hesse and by Rhine
    Princess Irene of Hesse and by Rhine
    Princess Irene of Hesse and by Rhine was the third child and third daughter of Princess Alice of the United Kingdom and Ludwig IV, Grand Duke of Hesse and by Rhine. Her maternal grandparents were Queen Victoria of the United Kingdom and Prince Albert...

     (later Princess Heinrich of Prussia), passed it on to two of her three sons:
    • Prince Waldemar of Prussia
      Prince Waldemar of Prussia (1889-1945)
      Prince Waldemar of Prussia was the eldest son of Prince Heinrich of Prussia and his wife, Princess Irene of Hesse and by Rhine.-Biography:-Marriage:...

      . Survived to age 56; had no issue.
    • Prince Heinrich of Prussia. Died at age 4.

  • Princess Alix of Hesse and by Rhine. Alix had a marriage proposal from Prince Albert Victor, eldest son of the future King Edward VII
    Edward VII of the United Kingdom
    Edward VII was King of the United Kingdom and the British Dominions and Emperor of India from 22 January 1901 until his death in 1910...

    ; had she accepted, haemophilia could have returned to the direct line of succession in Britain. Instead she married Tsar Nicholas II of Russia
    Nicholas II of Russia
    Nicholas II was the last Emperor of Russia, Grand Prince of Finland, and titular King of Poland. His official short title was Nicholas II, Emperor and Autocrat of All the Russias and he is known as Saint Nicholas the Passion-Bearer by the Russian Orthodox Church.Nicholas II ruled from 1894 until...

     and passed it on to her only son
    Tsarevich Alexei Nikolaevich of Russia
    Alexei Nikolaevich of the House of Romanov, was the Tsesarevich and heir apparent to the throne of the Russian Empire. In English, his title is usually given as Tsarevich, a title that has a separate meaning in Russia. Alexei was the youngest child and only son of Emperor Nicholas II and Empress...

    :
    • Tsarevitch Alexei. Murdered by the Bolshevik
      Bolshevik
      The Bolsheviks, originally also Bolshevists , derived from bol'shinstvo, "majority") were a faction of the Marxist Russian Social Democratic Labour Party which split apart from the Menshevik faction at the Second Party Congress in 1903....

      s at the age of 13, along with his parents and all four of his sisters. Alexei's haemophilia was one of the factors contributing to the collapse of Imperial Russia during the Russian Revolution of 1917
      Russian Revolution of 1917
      The Russian Revolution is the collective term for a series of revolutions in Russia in 1917, which destroyed the Tsarist autocracy and led to the creation of the Soviet Union. The Tsar was deposed and replaced by a provisional government in the first revolution of February 1917...

      . DNA testing of the Romanov family remains in 2009 showed that one of the four daughters, thought to be Maria by American researchers and Anastasia by Russian researchers, was a carrier. Grand Duchess Maria was thought by some to have been a symptomatic carrier because she haemorrhaged during a tonsillectomy
      Tonsillectomy
      A tonsillectomy is a 3,000-year-old surgical procedure in which the tonsils are removed from either side of the throat. The procedure is performed in response to cases of repeated occurrence of acute tonsillitis or adenoiditis, obstructive sleep apnea, nasal airway obstruction, snoring, or...

      .

  • Princess Victoria of Hesse and by Rhine
    Princess Victoria of Hesse and by Rhine
    Princess Victoria of Hesse and by Rhine, later Victoria Mountbatten, Marchioness of Milford Haven was the eldest daughter of Louis IV, Grand Duke of Hesse and by Rhine and his first wife Princess Alice of the United Kingdom .Her mother died while her brother and sisters...

     (later Victoria, Marchioness of Milford Haven), Alice's oldest child and maternal grandmother to Prince Philip, Duke of Edinburgh
    Prince Philip, Duke of Edinburgh
    Prince Philip, Duke of Edinburgh is the husband of Elizabeth II. He is the United Kingdom's longest-serving consort and the oldest serving spouse of a reigning British monarch....

    , might have inherited the mutation, though the gene remained hidden for several generations before possibly reappearing in the descendants of her eldest granddaughter, Princess Margarita of Greece and Denmark (see below).

  • Princess Elizabeth of Hesse and by Rhine
    Grand Duchess Elizabeth Fyodorovna
    Grand Duchess Elizabeth Feodorovna of Russia canonized as St. Elizabeth Romanova was a German princess of the House of Hesse, and the wife of Grand Duke Sergei Alexandrovich of Russia, fifth son of Emperor Alexander II of Russia and Princess Marie of Hesse and the 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
    Diphtheria
    Diphtheria is an upper respiratory tract illness caused by Corynebacterium diphtheriae, a facultative anaerobic, Gram-positive bacterium. It is characterized by sore throat, low fever, and an adherent membrane on the tonsils, pharynx, and/or nasal cavity...

     at the age of four.

Prince Leopold

Leopold
Prince Leopold, Duke of Albany
The Prince Leopold, Duke of Albany was the eighth child and fourth son of Queen Victoria and Prince Albert of Saxe-Coburg and Gotha. Leopold was later created Duke of Albany, Earl of Clarence, and Baron Arklow...

, 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):
  • Princess Alice of Albany
    Princess Alice, Countess of Athlone
    Princess Alice, Countess of Athlone was a member of the British Royal Family. She was the longest-lived Princess of the Blood Royal of the British Royal Family and the last surviving grandchild of Queen Victoria...

     (later Countess of Athlone), who in turn passed it on to her oldest son:
    • Prince Rupert of Teck (died at the age of 20, bleeding to death after a car accident)


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
Lady May Abel Smith
Lady May Abel Smith born Princess May of Teck was a descendant of the British Royal Family, a great-granddaughter of Queen Victoria. From her birth, she was known as Princess May of Teck, a title in the Kingdom of Württemberg...

, Leopold's granddaughter, has living descendants none of whom has been known to have or to transmit haemophilia.

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 four children:
  • Princess Victoria Eugenie of Battenberg (later Queen Victoria Eugenia of Spain), who passed it on to
    • Infante Alfonso of Spain, Prince of Asturias
      Alfonso of Spain, Prince of Asturias
      Alfonso, Prince of Asturias Alfonso, Prince of Asturias Alfonso, Prince of Asturias (Alfonso Pío Cristino Eduardo Francisco Guillermo Carlos Enrique Eugenio Fernando Antonio Venancio Borbón y Battenberg (Madrid, 10 May 1907 – Miami, Miami-Dade County, Florida, 6 September 1938), was an Infante of...

      . Died at age 31, bleeding to death after a car accident.
    • Infante Gonzalo
      Infante Gonzalo of Spain
      Infante Gonzalo of Spain was the fourth surviving son and youngest child of King Alfonso XIII of Spain and his wife Princess Victoria Eugenie of Battenberg....

      . Died at age 19, bleeding to death after a car accident.
    • Victoria Eugenie's two daughters, Infantas Beatriz
      Infanta Beatriz of Spain
      The Infanta Beatriz of Spain was a daughter of King Alfonso XIII of Spain and Victoria Eugenie of Battenberg and paternal aunt of the current King Juan Carlos I.-Early life:Infanta Beatriz was born at La Granja, San Ildefonso near...

       and Maria Cristina of Spain
      Infanta Maria Cristina of Spain
      Infanta Maria Cristina of Spain, Countess of Marone was the fourth child of Alfonso XIII of Spain and Victoria Eugenie of Battenberg and paternal aunt of the current King Juan Carlos I.-Early life:Infanta Maria...

      , both have living descendants none of whom has been known to have or to transmit haemophilia.
  • Prince Leopold of Battenberg (later, Lord Leopold Mountbatten). Died at age 32 during a knee operation.
  • Prince Maurice of Battenberg
    Prince Maurice of Battenberg
    Prince Maurice of Battenberg, KCVO, was a member of the Hessian princely Battenberg family and the extended British Royal Family, the youngest grandchild of Queen Victoria...

    . Killed in action in World War I
    World War I
    World War I , which was predominantly called the World War or the Great War from its occurrence until 1939, and the First World War or World War I thereafter, was a major war centred in Europe that began on 28 July 1914 and lasted until 11 November 1918...

     in 1914 at the age of 23. Maurice's haemophilia is disputed by various sources: It seems unlikely that a known haemophiliac would be allowed to serve in combat.

Today

No living member of the present or past reigning dynasties
Dynasty
A dynasty is a sequence of rulers considered members of the same family. Historians traditionally consider many sovereign states' history within a framework of successive dynasties, e.g., China, Ancient Egypt and the Persian Empire...

 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
Don (honorific)
Don, from Latin dominus, is an honorific in Spanish , Portuguese , and Italian . The female equivalent is Doña , Dona , and Donna , abbreviated "Dª" or simply "D."-Usage:...

 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.
  • 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 two eldest daughters, Beatrice and Sibilla
    Princess Sibilla of Luxembourg
    Princess Sibilla of Luxembourg , was born in Neuilly-sur-Seine, France on 12 June 1968.-Family:...

     are both married with children, none of whom, in the case of their sons, appear to be haemophiliacs. If Sibilla's descendants were to express or transmit the gene, however, another reigning dynasty of Europe would, in the 21st century, join the rest of the reigning families that inherited the disease from Queen Victoria. Olimpia's youngest daughters are still unmarried, but there is still a chance they could be carriers. Another daughter, Laura, died as a child, as did her only son, Paul, the latter of whom was apparently not a haemophiliac.
  • 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
    Don (honorific)
    Don, from Latin dominus, is an honorific in Spanish , Portuguese , and Italian . The female equivalent is Doña , Dona , and Donna , abbreviated "Dª" or simply "D."-Usage:...

     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.

Ferdinand Soltmann

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
House of Croÿ
The House of Croÿ is an international family of European mediatized nobility which held a seat in the Imperial Diet from 1486, and was elevated to the rank of Imperial Princes in 1594...

. 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. 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
Princess Alice of the United Kingdom
The Princess Alice was a member of the British royal family, the third child and second daughter of Queen Victoria and Prince Albert of Saxe-Coburg and Gotha.Alice's education was devised by Albert's close friend and adviser, Baron Stockmar...

 and Alfred, Duke of Saxe-Coburg and Gotha
Alfred, Duke of Saxe-Coburg and Gotha
Alfred, Duke of Saxe-Coburg and Gotha was the third Duke of Saxe-Coburg and Gotha, and reigned from 1893 to 1900. He was also a member of the British Royal Family, the second son and fourth child of Queen Victoria and Prince Albert 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 Alice of the United Kingdom
The Princess Alice was a member of the British royal family, the third child and second daughter of Queen Victoria and Prince Albert of Saxe-Coburg and Gotha.Alice's education was devised by Albert's close friend and adviser, Baron Stockmar...

 
 
 Princess Victoria of Hesse and by Rhine
Princess Victoria of Hesse and by Rhine
Princess Victoria of Hesse and by Rhine, later Victoria Mountbatten, Marchioness of Milford Haven was the eldest daughter of Louis IV, Grand Duke of Hesse and by Rhine and his first wife Princess Alice of the United Kingdom .Her mother died while her brother and sisters...

 
 Princess Alice of Battenberg
Princess Alice of Battenberg
Princess Alice of Battenberg, later Princess Andrew of Greece and Denmark was the mother of Prince Philip, Duke of Edinburgh, and mother-in-law of Elizabeth II....

 
 Princess Margarita of Greece and Denmark
 
 Kraft, 9th Prince of Hohenlohe-Langenburg
Kraft, Prince of Hohenlohe-Langenburg
Kraft, Prince of Hohenlohe-Langenburg was the eldest son of Gottfried, Prince of Hohenlohe-Langenburg. He was the titular Prince of Hohenlohe-Langenburg from 1960 until his death.-Early life:...

 
 Princess Xenia of Hohenlohe-Langenburg
 
 Ferdinand Soltmann

Chronological order

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.
Men who died of Haemophilia in Order of Death
# Name DeathRelation 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

Type of haemophilia discovered

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
Haemophilia B
Haemophilia B is a blood clotting disorder caused by a mutation of the Factor IX gene, leading to a deficiency of Factor IX. It is the second most common form of haemophilia, rarer than haemophilia A. It is sometimes called Christmas disease after Stephen Christmas, the first patient described...

.
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