What is haemophilia
It is an X-linked genetic disease, hence the genetic
abnormality is found on the X chromosome.
There are two types heamophilia A and
B.Haemophilia A has a deficit in factor viii and B in
It usually presents in infancy with recurrent bleeds,
,painful haemarthoses and haematomas and
Bleeding episodes are treated with Factor viii or ix
replacement. DDAVP is also used
The Earliest written recognition of hemophilia was in
2nd century AD in Jewish writings
Females are usually carriers, Since they have a
chromosomal make-up of XX, inheriting an X
chromosome with disease mutations will generally
not produce the disease condition as the other normal
X chromosome can compensate.
Hemophilia affects males much more frequently (1 in
10,000) than females (1 in 100,000,000).
Spontaneous mutation can occur . In 33% of patients
there is no family history of haemophilia
“The royal disease”
haemophilia became known as the
“Royal disease” because it spread
to the royal families of Europe through
Haemophilia appeared in Queen Victoria’s
children, and through them spread into the
royal families of Europe
Queen Victoria had 9 children. One
hemophilic son and two daughters that
Queen Victoria had no ancestors with the
condition but soon after the birth of her
eighth child, Leopold, in 1853 it became
evident that he had haemophilia (the son
Leopold died at the age of 31, from a intra
cerebral haemorrhage and was described as
With the appearance of hemophilia in the
royal family the Queen could only protest
that the disease did not originate in her side
of the family.and they often blamed the
“curse of the Coburgs”.
How did the gene get introduced into
the royal family
There were several suggestions how the
haemophilic gene entered the royal family
1 .spontaneous mutation
2.and the most controversial : that queen Victoria
wasn’t the Duke of Kent’s daughter.
The most accepted theory is that of spontaneous
mutation ,as 30% of cases represents with new
mutations, it is more likely that Victoria's carrier
state was due to a new germ line mutation in her
parents or grandparents
The haemophilic gene has now died out in these
Royal families, Thus we do not know to this day if
the condition was haemophilia A or B
Affecting European royalty
Queen Victoria was known as a
successful dynasty as her children
and grandchildren married widely
among the powerful ruling families
Hence the haemophilic gene spread
through the royal houses of Europe
as monarchs arranged marriages to
consolidate political alliances .
Hence the Russian , Spanish and
German royal families were affected.
( as two of the daughters of Queen
Victoria were carriers)
The Russian connection
Alix of Hesse was one of Victoria’s grand daughters and
married Tsar Nicholas II of Russia, becoming Tsarina
she was a carrier and her fifth child and only son, Alexi, was
born in 1904. He was a haemophiliac
Alexi’s hemophilia discovered shortly after birth because of
excessive bleeding from his umbilicus
Rasputin got involved with the Romanovs when Alexei
got a bruise after falling off a horse and the tsarina was
looking everywhere for help. And Rasputin was
suggested by her best friend as he was known to be a
“charismatic peasant healer”.
He seemed to be the only one able to soothe Alexei
It is still unsure how Rasputin was able to soothe the
child. It is believed that he hypnotised the boy.
Rasputin was a very unpopular and controversial figure,
leading a scandalous personal life. His connection with
the Romanovs stained the royal family
Rasputin was given much authority by Alexandra because
of his “healing powers”, and he was thus able to influence
politics and decision makingHe soon became her
confidant and personal advisor. He also convinced her to
fill some government offices with his own handpicked
This reliance enhanced Rasputin's political power, which
seriously undermined the Romanov rule during the First
At this point Russia was in turmoil
Alexandra was unpopular in Russia during
the first world war, as her connection with
the German royalty and she was thought to
be a spy.
The Russian Revolution began in 1914. Tsar
Nicholas II was not a strong leader, and his
son’s illness only added to his inability to rule
his country by making the family more
sheltered and out of touch with the people
Nicholas II abdicated in 1917
The family was executed on July 17, 1918.
Many historians attribute much of the Tsar’s
fall to his son’s illness and his inability to
take more effective steps and there is little
doubt of the influence of Rasputin.
The German connection
Queen Victoria’s grand-daughter Irene of
Hesse, Princess of Prussia introduced the
gene into the German imperial families
She had three sons, two of whom were
Spanish royal family
Princess Victoria Eugenie Battenberg of
England , Queen Victoria's grand daughter
married King Alfonso XIII of Spain
She gave birth to 7 children , 2 haemophilic
sons and only one healthy son.
2 sons had haemophilia, one son was stillborn, the second
eldest son was death and mute
This news of haemophilia damaged Victoria Eugenia's relation
with her husband. It also sent the King into a tailspin of
irresponsible behaviour that led him to abandon the affairs of
daily government to questionable characters and dictators .
this also weakened the position of monarchy in Spain and
discredited the heirs and may have lead to the civil war.
It is impossible to measure exactly the effect of these tragedies
on the Spanish throne.The Spanish society was so deeply
divided and flawed that civil war might have been
unavoidable, but undoubtedly helped to weaken the position of
A particular slide catching your eye?
Clipping is a handy way to collect important slides you want to go back to later.