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The Family Of Queen Victoria
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Haemophilia In The
Descendants Of
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Albert Victor And
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 Haemophilia in the Descendants of
Queen Victoria

Haemophilia acquired the

name the royal disease due to the high number of descendants of Queen
Victoria afflicted by it. The first instance of haemophilia in the British Royal
family occured on the birth of Prince Leopold on 7th April, 1853, Leopold was
the fourth son and eighth child of Queen Victoria and Prince Albert of Saxe-
Coburg-Gotha. No earlier occurence of the disease in the Royal family had
been known, it is assumed that a mutation occured in the sperm of the
Queen's father, Edward Augustus, Duke of Kent.
 Haemophilia is an X-linked recessive
disorder. The blood of a haemophiliac lacks the ability to coagulate, due to the
fact that one or more of the plasma proteins required to form a clot is absent
or reduced in their blood. The condition is passed on to males through
females, who do not manifest the symptoms of the disease themselves. A
recessive gene, it is carried on the sexual female chromosome X . Males
possess XY chromosomes and females XX. Since females have two X
chromosomes, they are more often than not carriers.

Prince Leopold, Duke of Albany (1 on chart), the first of Queen Victoria's

descendants to suffer from haemophilia, was described as delicate child who
remained a constant source of anxiety to the Queen throughout his life,
evidence exists that Leopold also suffered mildly from epilepsy, like his grand-
nephew Prince John (the youngest son of King George V). He was first
diagnosed with haemophilia in 1858 or 1859, Queen Victoria consequently
placed restrictions on him, which he chaffed at. He was later created Duke of
Albany and married the German princess, Helena of Waldeck-Pyrmont.
Leopold died in 1884 at the age of 31, in the south of France. He suffered a fit,
the cause or the consequence of a fall on some stairs at Cannes, injuring his
knee and hitting his head and died the following morning, apparently from a
cerebral haemorrhage.
 Leopold was the only one of Queen Victorias haemophiliac descendants
to have children, his marriage to Helena of Waldeck produced two children, a
daughter, Princess Alice of Albany (4), later to become Countess of
Athlone, who was a further carrier of the disease and an unaffected son, born
posthumously, Charles Edward, later Duke of Saxe-Coburg-Gotha. Alice was
later to become Countess of Athlone and was to prove a carrier of
haemophilia. She married Prince Alexander of Teck, the brother of Queen
Mary, their son, Rupert Alexander George of Teck, Viscount Trematon
(5) was also a haemophiliac. He died on 15 April 1928 from an intracerebral
hemorrhage as a result of a car crash in France.

During the First World War, when anti-German feeling was at its height, in
conjunction with changing the name of the Royal House to Windsor, King
George V changed that of the Tecks to Cambridge, (for their maternal
ancestor, Adolphus, Duke of Cambridge, a son of George III). Alexander of
Teck was made Earl of Athlone and Rupert granted the courtesy title of
Viscount Trematon. After being involved in a car accident in France, he died
of a brain hemorrhage.
 Through two of the Queen's daughters, Alice (2) and Beatrice (3), both of
whom were carriers, the disease was to be spread into many of the Royal
Families of Europe.

Princess Alice was married to Prince

Louis of Hesse-Darmstadt and gave birth to a haemophiliac son, Frederick of
Hesse (6), known as Frittie in the family, in 1870. He died very young in 1873,
after a fall from a window induced a hemorrhage. Tragically, the child literally
bled to death, leaving his mother inconsolable. Alice also had an unaffected
son, the future Grand Duke Ernest Louis of Hesse and five daughters. Two of
the daughters, Irene (7) and Alix of Hesse(8) were in turn, carriers of the
haemophilia gene.

Haemophilia appeared in the Prussian Royal family, when Alice's third

daughter Irene married her first cousin, Prince Henry of Prussia, the second
son of Queen Victoria's eldest daughter Victoria, Princess Royal and brother
of Kaiser Wilhelm II. The disease appeared in two of their sons
PrincesWaldemar (9) and Henry of Prussia (10).

Prince Waldemar died in a clinic in Tutzing, Bavaria during the Second

World War due to a lack of blood transfusion facilities. He and his wife fled
before the Russian advance, arriving in Tutzing, Waldemar was in need of a
blood transfusion but the U.S. Army overran the area and diverted all
available medical resources to treat concentration camp victims, preventing
Waldemar's German doctor from treating him, Waldemar died the following
day, on 2 May, 1945. His brother Prince Henry died at the age of four on 26
February 1904, from a brain haemorage, the result of a fall from a chair.

The disease was spread to the Romanov dynasty through the marriage of
Alice's fourth daughter Alix, to Tsar Nicholas II, at which she became the
Empress Alexandra of Russia. The highly attractive Alix had previously
refused a proposal from Albert Victor, Duke of Clarence and Avondale, and
heir to the British throne, the eldest son of Bertie, Prince of Wales. Had she
accepted, haemophilia could have re-entered the British Royal line. Nicholas
had long loved and cherished dreams of marrying Alix, but she turned down
his first proposal as she could not bring herself to change her Protestant
religion to the Russian Orthodoxy required of a future Tsarina, but after much
soul searching, accepted when Nicholas proposed for a second time.

Alix, who became known as the

Empress Alexandra, produced four daughters before giving birth to their only
son, theTsarevitch Alexis (11), heir to the Russian empire, who was also
stricken with haemophilia. As with most mother's of haemophiliacs, Alix was
overprotective of her son and worried about him constantly. Through his
supposed ability to heal the Tsarevich, and the Tsarina's compete confidence
in him, Rasputin acquired a fatal influence over the Tsar's decisions which
lead directly to the Russian Revolution. The entire family perished at the
hands of a Bolshevik firing squad in a cellar at Ekaterinberg on 17th July,
1918.

The Queen's youngest

daughter, Princess Beatrice, fell in love with and married the handsome
Prince Henry of Battenberg. The couple produced three sons and a daughter.
Two of their sons, Leopold Mountbatten (12) and Maurice, Prince of
Battenburg (13) inherited the haemophilia gene from their mother. Maurice
was killed whilst engaged in active service in the Ypres Salient during the First
World War. Leopold lived to the age of 32, dying during a hip operation in
1922.

Beatrice's only daughter, Victoria Eugenie of Battenburg (14), known as

Ena, was married to King Alfonso XIII of Spain and carried the disease into
the Royal House of Spain.
 Though they did not enjoy a particularly happy marriage and Alfonso had
many mistresses, the couple produced six children, four sons and two
daughters. Two of their sons, Alfonso, Prince of the Asturias (15), the heir
to Spain, and Infante Gonzalo of Spain (16), were affected with haemophilia.
Alfonso is reported to to have never forgiven his wife for passing the disease
into the Spanish Royal bloodline. Both children were dressed in padded suits
to prevent their undergoing knocks which might result in a life threatening
hemorrhage.

Alfonso later renounced his rights to the throne of Spain to marry a

commoner, Edelmira Sampedro Ocejo y Robato. A car accident led to his
early death in 1938. Another of Victoria Eugenie's sons Juan, was the father
of Juan Carlos, the present King of Spain. In August 1934 Gonzalo was
spending the summer holidays with his family at the villa of Count Ladislaus
Hoyos at Prtschach am Wrthersee in Austria. The infante Gonzalo died as
a result of a traffic accident, he and his sister the Infanta Beatriz were driving
from Klagenfurt to Prtschach. On approaching Krumpendorf, Beatriz, who
was driving the vehicle, was forced to swerve to avoid a cyclist, resulting in
the car being crashed into a wall. Since neither Gonzalo nor Beatriz appeared
badly hurt, they returned to their villa. Several hours later it became clear that
Gonzalo had severe abdominal bleeding and died two days later.

Porphyria in the Royal Family

On to the family of Queen Victoria -->

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Red-green color blindness is due to an X-linked recessive allele in humans. A widow's
peak is due to an autosomal dominant allele. Consider the following family history:
* A man with a widow's peak and normal color vision marries a color-blind woman with a
straight hairline.
* The man's father had a straight hairline, as did both of the woman's parents.
Use the family history to make predictions about the couple's children.
If the couple has a child, what is the chance that it will be a son with a widow's peak?
1/4

Red-green color blindness is due to an X-linked recessive allele in humans. A widow's

peak is due to an autosomal dominant allele. Consider the following family history:
* A man with a widow's peak and normal color vision marries a color-blind woman with a
straight hairline.
* The man's father had a straight hairline, as did both of the woman's parents.
Use the family history to make predictions about the couple's children.
What is the chance that any son the couple has will be color blind with a straight
hairline?
1/2

Red-green color blindness is due to an X-linked recessive allele in humans. A widow's

peak is due to an autosomal dominant allele. Consider the following family history:
* A man with a widow's peak and normal color vision marries a color-blind woman with a
straight hairline.
* The man's father had a straight hairline, as did both of the woman's parents.
Use the family history to make predictions about the couple's children.
What is the chance that any daughter that the couple has will be color blind with a
widow's peak?
0

Red-green color blindness is due to an X-linked recessive allele in humans. A widow's

peak is due to an autosomal dominant allele. Consider the following family history:
* A man with a widow's peak and normal color vision marries a color-blind woman with a
straight hairline.
* The man's father had a straight hairline, as did both of the woman's parents.
Use the family history to make predictions about the couple's children.
Suppose the couple had a daughter with normal color vision and a widow's peak. What
is the chance that she is heterozygous for both genes?
1

In humans, what determines the sex of offspring and why?

a) The chromosome contribution from both parents determines sex because the
offspring uses all the parents' chromosomes.
b) The female gamete determines sex because only the female gamete provides
cytoplasm to the zygote.
c) The male determines sex because the sperm can fertilize either a female egg or a
male egg.
d) The male gamete determines sex because each male gamete can contribute either
an X or a Y chromosome.
e) The female gamete determines sex because only the female gametes can have one
of the two functional sex chromosomes.
d) The male gamete determines sex because each male gamete can contribute either
an X or a Y chromosome.

Which of the following is true of an X-linked gene, but not of a Y-linked gene?
a) It is expressed in half of the cells of either male or female.
b) It does not segregate like other genes.
c) The gene is present in both males and females.
d) Sister chromatids separate during mitosis.
e) It is only expressed in female offspring.
c) The gene is present in both males and females.

In general, the frequency with which crossing over occurs between two linked genes
depends on what?
a) whether the genes are dominant or recessive
b) whether the genes are on the X or some other chromosome
c) how far apart they are on the chromosome
d) the characters the genes code for
e) the phase of meiosis in which the crossing over occurs.
c) how far apart they are on the chromosome

What is a nondisjunction?
a) An error in which a diploid cell or organism has an extra chromosome of one type,
producing a chromosome number of 2n+1
b) An error in cell division that causes homologous chromosomes or sister chromatids
to move to the same side of the dividing cell
c) None of the above
d) An error in which a diploid cell or organism lacks a chromosome of one type,
producing a chromosome number of 2n-1.
b) An error in cell division that causes homologous chromosomes or sister chromatids
to move to the same side of the dividing cell

When can nondisjunction occur? Choose the best answer.

a) In mitosis, when sister chromatids fail to separate
b) In meiosis, when homologous chromosomes fail to separate
c) In meiosis, when sister chromatids fail to separate
d) All three answers are correct
d) All three answers are correct

Which syndrome is characterized by the XO chromosome abnormality?

a) Klinefelter syndrome
b) Trisomy X
c) Turner syndrome
d) Down syndrome
c) Turner syndrome

What kind of cell results when a diploid and a haploid gamete fuse during fertilization?
a) A monoploid cell
b) A trisomic cell
c) A triploid cell
d) A monosomic cell
c) A triploid cell

Of the following chromosomal abnormalities, which type is most likely to be viable in

humans?
a) Haploidy
b) Monosomy
c) Trisomy
d) Triploidy
c) Trisomy

If a diploid cell undergoes meiosis and produces two gametes that are normal, and one
with n-1 chromosomes, and one with n+1 chromosomes, what type of error occurred?
a) A nondisjunction error occurred in meiosis I, in which both members of a homologous
pair migrated to the same of the cell.
b) No error occurred; these are normal gametes
c) A nondisjunction error occurred in meiosis I, in which both members of all
homologous pairs migrated to the same pole of the cell.
d) A non disjunction error occurred in meiosis II, in which both sister chromatids of a
chromosome migrated to the same pole of the cell.